Vol.5 No.2

2003; 5(2):89
INDIAN JOURNAL OF IJPP

PRACTICAL PEDIATRICS
A quarterly medical journal committed to practical pediatric problems and
management update presented in a readable manner
IJPP is a subscription Journal of the Indian Academy of Pediatrics
Indexed in Excerpta Medica dated since January 2003
Annual subscription:Rs. 300/- 10 years subscription:Rs.3000/-
Vol.5 No.2 APRIL-JUNE 2003

Dr. A. Balachandran Dr. D.Vijayasekaran
Editor-in-Chief Executive Editor
CONTENTS
FROM THE EDITOR'S DESK 91
TOPIC OF INTEREST - LABORATORY MEDICINE
Editorial - Guidelines for collection of specimens for microbiological
investigations 93
- Elizabeth Mathai
Interpretation of laboratory investigations in the diagnosis of
intrauterine infections 99
- Indrashekar Rao
Diagnostic approach to the child with arthritis 105
- Ramanan AV, Akikusa JD
Rapid diagnostic tests - Benefits and Pitfalls 111
- Saranya N
Neonatal Metabolic screening tests 118
- Nair PMC
Laboratory diagnosis of persistent and chronic diarrhoea 125
- Shinjini Bhatnagar
Journal Office : Indian Journal of Practical Pediatrics, IAP - TNSC Flat, F Block, Ground Floor, Hall Towers,
56, Halls Road, Egmore, Chennai - 600 008. INDIA. Tel No.: 044-28190032 E.mail : ijpp_iap@rediff mail.com
Address for registered / insured / speed post / courier letters / parcels and communication by various
authors : Dr. A. Balachandran, Editor-in-Chief, Indian Journal of Practical Pediatrics, F Block, No. 177, Plot
No. 235, 4th Street, Anna Nagar East, Chennai - 600 102. Tamil Nadu, INDIA.
1
Indian Journal of Practical Pediatrics 2003; 5(2):90
Laboratory evaluation of hypertension 133

- Vijayakumar M, Prahlad N, Nammalwar B.R.
Clinical Neurophysiology in Pediatric Practice 141
- Ayyar SSK, Suresh Kumar, Vasanthi D, Sangeetha V
NUTRITION
Smart nutrients and brain development 149
- Elizabeth K E
IJPP - IAP CME, 2001
Recognition of a critically ill child 154
- Ramachandran P
RADIOLOGIST TALKS TO YOU
Obstruction in urinary tract 159
- Vijayalakshmi G, Natarajan B, Ramalingam A
CASE STUDY
Dengue haemorrhagic fever in a boy with Bombay blood group -
A rare coincidence 163
- Janani Shankar, Adhisivam B
Caffeys disease (Infantile cortical hyperostosis) 165
- Mohammed Thamby, Nagarajan M, Ram Saravanan R
Neurofibromatosis type I with congenital pseudoarthrosis and
holoprosencephaly 167
- Preetha Prasannan, Veerendra Kumar, Jayakumar, Sukumaran TU
GLOBAL CONCERN
Severe acute respiratory syndrome (SARS) 170
PRACTITIONERS COLUMN
Tips for practising pediatrician 173
- Kamlesh R. Lala, Mrudula K.Lala
QUESTIONS AND ANSWERS 179
NEWS AND NOTES 92,98,104,132,140,153,158,180,182
FOR YOUR KIND ATTENTION
* The views expressed by the authors do not necessarily reflect those of the sponsor or
publisher. Although every care has been taken to ensure technical accuracy, no responsibility is
accepted for errors or omissions.
* The claims of the manufacturers and efficacy of the products advertised in the journal are the
responsibility of the advertiser. The journal does not own any responsibility for the guarantee of the
products advertised.
* Part or whole of the material published in this issue may be reproduced with
the note "Acknowledgement" to "Indian Journal of Practical Pediatrics" without prior permission.
- Editorial Board
2
2003; 5(2):91
EDITORS DESK
We are pleased to bring out the issue on rich knowledge and vast experience in their
Laboratory Medicine, which will be of immense articles for the benefit of our readers.
use to all our readers.
In the current scenario of increasing
This issue was meticulously formulated by consumer awareness about the laboratory
Dr. S. Thangavelu, Asst. Professor, Pediatric investigations, this issue will be of immense help
Intensive Care Unit (PICU), Institute of Child to the practicing pediatricians while ordering
Health and Hospital for Children, Chennai. He investigations in the management of day to day
has carefully chosen the topics which are relevant problems in clinical practice.
to all the practising pediatricians, academicians
and postgraduates. We are thankful to him for We have also included an article on Severe
his sincere effort in bringing out this interesting Acute Respiratory Syndrome (SARS) for the
and informative issue. benefit of our readers to highlight the salient
features of the recent outbreak of this disease.
The new strides in the day to day
developments in laboratory medicine need The third and fourth issue of IJPP for this
periodic review by experts in the field. year will be on Infectious diseases and HIV
infection respectively.
The editorial by Dr. Elizabeth Mathai is very
informative for all those engaged in clinical We welcome the comments and suggestions
practice. She has given the guidelines for from the members for further betterment of the
collection of specimens for microbiological journal. We also invite you to share the problems
investigations. We thank all the authors who have faced in clinical management by practising
contributed articles to this special issue in their pediatricians. This will be discussed by experts
respective field of interest. They have shared their in the journal.
Published and owned by Dr. A. Balachandran, from Halls Towers, 56, Halls Road,
Egmore, Chennai - 600 008 and Printed by Mr. D. Ramanathan, at Alamu Printing Works,
9, Iyyah Mudali Street, Royapettah, Chennai - 600 014. Editor : Dr. A. Balachandran.
3
NEWS & NOTES

INFANT AND YOUNG CHILD NUTRITION CONFERENCE
Theme: Men in Child nutrition and Care
Organised by
IAP Tamilnadu State Chapter, IAP-Chennai City Branch,
Breastfeeding Promotion Network of India & Nutrition Society of India
Date : 13th July, 2003
Venue : Hotel Vijay Park, 12 Jawahar Lal Nehru Salai, Inner Ring Road,
Arumbakkam, Chennai 600 106. Ph. No. 23791314
Near Hotel Radha Park Inn
Delegate Fee: Rs.300/- PG Student: Rs.250/-
Preconference Lactation Management Course : Date: 12th July, 2003
Venue: ICH & HC and M.H., Egmore, Chennai 600 008.
Registration Fee: Rs.200/- (Limited to 40 delegates only)
Last Date for registration: 5th July, 2003
Cheque / DD should be drawn in favour of CHEN NUTRICON 2003.
For outstation cheque please add Rs.50/-.
For registration contact
Dr.D.Gunasingh
Organising Secretary, Infant and Young Child Nutrition Conference
IAP Flat, F block, Halls Tower, 56 (Old No33), Halls Road, Egmore, Chennai 600 008.
Ph. No.044-28191524 Email: [email protected]
1ST NATIONAL CME ON INFECTIOUS DISEASES IN CHILDREN:

FROM PROBLEMS TO SOLUTION
Organised by : IAP Infectious Diseases Chapter
Venue: Asutosh Birth Centenary Hall: Indian Museum, Kolkata,
Date: 17th, 18th May 2003
Registration Fee Upto 28.02.2003 Upto 30.04.2003 Spot
Delegate Rs.500/- Rs.600/- Rs.750/-
Postgraduate Rs.400/- Rs.500/- Rs.600/-
DD or Cheque to be drawn in favour of IAP INFECTIOUS DISEASES CHAPTER
payable at Kolkata. Rs.50/- to be paid extra for outstation cheque. No cheque will be accepted
after 15.04.2003
Address for correspondance: Dr.Tapan Kr. Ghosh, Organising Chairperson, National CME
on Infectious Diseases in Children, 13, Neogi Pukur Bye Lane, Kolkata 7000 014. Ph: 033-22445551/
22164351, Mobile: 9831179718. Email: [email protected]
4
2003; 5(2):93
EDITORIAL
GUIDELINES FOR where the pathogen is most likely to be found

COLLECTING SPECIMENS (usually the infected site) and at a stage when
FOR MICROBIOLOGICAL organisms may be found in maximum numbers.
INVESTIGATIONS (S.typhi is most likely isolated from blood during
the first week of illness).
The most important factor that determines
the accuracy of any report is the quality of the 2. Care must be taken to send adequate quantity
sample received for testing. A wrong sample can of specimen as adequate material is required for
not only miss the pathogen but also lead to wrong making smears and performing cultures on
therapy. For example, a badly collected sputum multiple media. If different types of cultures
sample can lead to identification of members of (e.g., fungal, anaerobic, mycobacterial) are
commensal flora as pathogens resulting in wrong required, additional material should be sent.
choice of antibiotics. Therefore, it is important 3. Collect samples taking care to avoid/minimize
that all individuals involved in patient care should contamination with normal flora. In certain
understand the critical nature of maintaining situations like, swabs from burn wounds,
specimen quality. decubitus ulcer, etc it is impossible to avoid
Diagnostic tests offered by microbiology contamination with normal flora. In these cases,
laboratories include cultures to isolate the care should be exercised in interpreting culture
pathogen, immunological tests to detect antibody reports. It is also advisable that items like Foleys
response or antigens and more recently, catheter tips, which are likely to contain a variety
molecular techniques to detect nucleic acids of of colonizing organisms, are not sent for culture.
the suspected pathogen. In India, however, 4. Antibiotic therapy can make organisms
laboratories mostly offer bacterial and fungal nonviable or decrease the numbers. Therefore,
cultures and serology only. Therefore some collect samples for bacterial culture prior to
important guidelines for collecting specimens for initiating antibiotic therapy. In limited conditions,
these investigations are presented. for example, infection with bacteria resistant to
The sample received in the laboratory should the antibiotic being used or when there is lack of
ideally have the original numbers and proportions penetration of antimicrobial to the site of
(or close to it) of bacteria as in the infected site, infection, bacteria can still be isolated from
in a viable state. In order to achieve this, one has infected sites. While interpreting culture results
to choose the appropriate anatomical site for take this information into consideration.
sampling, collect and transport the sample 5. For culture, always use sterile leak proof
properly. It is helpful to remember the following containers that can be closed tightly. Do not fill
general principles in this regard. to the brim and always avoid soiling of the
exterior with sample while collecting and
1. The sample has to be appropriate and
transporting. Avoid soaking swabs in saline/
representative. Collect samples from the site
distilled water prior to specimen collection.
5
6. Label the container clearly with patient therapy and transported immediately to the
identification details and send the sample to the laboratory, to visualise motile spirochaetes.
laboratory along with the request slip detailing
the nature of infection, type of sample, time of 2. Pyogenic infections like wound and burns
collection and any other pertinent information. infection / ulcers / abscesses / cellulitis
Adequate clinical information will help the The most appropriate specimen to diagnose
microbiologist to assess the sample and modify a pyogenic infection is aspirated pus or biopsy
processing for special needs. of the infected tissue. Since this is not always
7. All samples are to be considered potentially practical, swabs of the infected site are used.
infective. It is therefore mandatory that Swabs however are not suitable for anaerobic
appropriate precautions are taken like wearing culture and culture for fungus.
of gloves and mask are taken while handling Prior to swabbing do not apply antiseptics.
samples. If there are crusts, these can be removed using
8. In general, transport without delay and without sterile distilled water or sterile saline. Be
additives. absolutely sure that saline/distilled water is sterile.
Using two swabs collect adequate pus or exudate
1. Urinary tract infection (UTI) by allowing sufficient contact by rolling the swab
over the infected site. If exudate is absent, rub
UTI is diagnosed by demonstrating infected the floor and margins of the infected areas with
urine in the bladder. The most reliable method the swab. Do not wet swabs with saline/distilled
of achieving this is by culture of a representative water.
sample of urine.
If there is an abscess, aspirate the pus and
To avoid contamination with perineal and transfer into a sterile plain (no additives) tube
urethral flora, midstream clean catch urine is with tight fitting stopper. The column of pus
collected directly into a wide mouth sterile will also allow anaerobic conditions to be
container. Since this is a specimen collected by maintained for a period of time. Biopsies should
the patient, clear instructions should be given be sent in sterile plain (no additives) container
regarding method of collection; children need to as early as possible to the laboratory. If the tissue
be supervised when samples are collected. In is very small and liable to drying, transport in a
babies and children who cannot co-operate, urine small amount of sterile (make absolutely sure)
may be collected by suprapubic aspiration. Urine saline.
may be collected by aspiration from long term
indwelling catheters after cleaning the site of Transport without delay. If delay is
aspiration with disinfectant. Catheter tips are not inevitable the sample can be refrigerated for a
good samples for diagnosing UTI and should be maximum period of 4 to 6 hours. Samples for
avoided. anaerobic culture should not be refrigerated.
Urine for culture should reach the lab within 3. Fluids
two hours. If this cannot be achieved, urine can
be refrigerated up to a maximum of 4 to 6 hours. Fluids from normally sterile areas like the
CSF, ascitic fluid, pleural fluid, synovial fluid
Urine for dark field microscopy for lepto- etc should be sent in sterile containers. If they
spirosis should be collected before antimicrobial are liable to clot, collect in containers with
6
2003; 5(2):95
anticoagulants such as 2.5% sodium citrate. Do 5. Acute lower respiratory infections (LRI)
not submit swabs dipped in fluid for culture.
The most frequently sent sample for the
Do not refrigerate these samples since some diagnosis of LRI is sputum. However, the value
of the potential pathogens are temperature of sputum culture is limited by the inability to
sensitive. avoid contamination with oral flora.
4. Upper respiratory infection. Rinse the mouth prior to collection, with
water, a few times. Encourage the patient to
Collect respiratory samples taking care to cough deeply and collect the coughed out material
minimize contamination with normal flora. directly into a wide mouthed sterile bottle with
Throat swab: Collect throat swab under proper screw cap. Do not add saliva into this. Early
vision. Depress the tongue and make the patient morning sample, collected during the first bout
say a long ah. Using two swabs rub well over of cough after waking up, is best for isolating
tonsils, the tonsillar fossa on both sides and lastly pathogens. For children, sputum collection should
the posterior pharyngeal wall. In addition, collect be done under the supervision of a trained person.
any obvious exudate. Withdraw the swabs Send the sample without delay and without
without touching tongue and cheek. Place the additives. Refrigeration can affect the recovery
swab in a sterile container without additives. of pathogens like H.influenzae
Drying of the swab will not affect the recovery
of beta haemolytic streptococci. In the laboratory, sputum is assessed for
quality. A good quality specimen will have large
If membrane like lesions are present, number of pus cells with very few squamous
collect a bit and send for culture. It is extremely epithelial cells.
important in this situation, to give the laboratory
the relevant clinical information. Since the agents of bacterial pneumonia like
S. pneumoniae and H .influenzae can be normal
Throat and nasopharyngeal swabs are not commensals in the upper respiratory tract, care
useful for identifying the aetiogical agents of has to be taken while interpreting results. It should
sinusitis, or acute otitis media. Culture is not also be remembered that bacterial respiratory
required on a routine basis for these infections. pathogens like Mycoplasma pneumoniae,
Treatment can be started empirically since most Chlamydiae pneumoniae and Legionella
infections are caused by a few limited pathogens. pneumophila will not grow in routine sputum
For resistant sinusitis, sinus pus should be culture and cannot be identified using Gram stain.
cultured. To identify the cause of otitis, collect These infections are currently diagnosed using
fresh discharge (if present) under vision and after non cultural methods like immuno-diagnosis and/
cleaning the crusts away using sterile saline/ or PCR.
distilled water. If there is no discharge,
tympanocentesis may be required. M.tuberculosis will also not grow in routine
culture and cannot be seen in Gram stain.
Nasal swabs are taken if a staphylococcal Therefore a special request should be made if
carrier state is suspected. Nasopharyngeal pulmonary tuberculosis is suspected.
sampling requires special swabs and is indicated
mostly in detecting carrier states (e.g. Nosocomial pathogens are survivors and
meningococci). will grow on routinely used media.
7
Agents of pneumonia in an immuno- area again, without sterile gloves. Collect

compromised patient may be different from those adequate quantity of blood using a sterile dry
in a normal host. Therefore, this information syringe and inoculate directly into blood culture
should be given to the laboratory and tests for media, using the same needle. Several
agents like P.carinii, requested separately if commercial media are now available for blood
indicated. culture. Additional media may be needed for
fungal and mycobacterial culture. Blood to
Broncho-alveolar lavage (BAL) collected medium ratio can range from1:10 to 1:5.
properly is a better sample than sputum. However
contamination with normal flora can not be ruled Warm the media to room temperature prior
out in this sample also. to inoculation. Do not refrigerate after
inoculation. Multiple blood cultures may be
6. Infections in the eye required for the diagnosis of endocarditis.
Corneal ulcers require microbiological Bone marrow cultures may yield better
investigation. Corneal scraping is the best sample results in situations like typhoid fever and
and it is advisable that this is done by an brucellosis. These may be sent to the laboratory
ophthalmologist. Since the sample is extremely or directly inoculated into blood culture medium.
small and organisms likely to be fastidious, the
sample is best inoculated at the site of collection Specimens for Mycobacteria smear and
itself on to media for isolation - Blood agar and culture
Chocolate agar for bacteria; Sabourauds
dextrose agar for fungi. If acanthamoeba keratitis Collect specimens from the site of infection
is suspected, the medium for this culture also as for routine culture.
should be included. Extra-material may be used Sputum is the recommended sample for
for microscopy. Sample both eyes separately. diagnosing pulmonary tuberculosis. Since the
7. Faeces sensitivity of sputum examination is low, the test
has to be repeated at least three times. An
Culture is not required on a routine basis alternative to sputum is fasting gastric juice,
for the management of diarrhea/dysentery. aspirated early in the morning. The entire amount
should be sent in a sterile container within 30
If culture is indicated, collect the sample minutes. Delay can make the organisms non-
directly into a sterile wide mouthed container with viable.
screw cap or snap on cap. Samples mixed with
urine and water are not suitable. Likelihood of a positive report from fluids
increase with the quantity sampled. Therefore
Clean containers are sufficient for send as much as possible in sterile containers, to
parasitological examination. enable the laboratory to concentrate the material.
8. Blood For renal tuberculosis, send the entire early
Blood for culture should be drawn under morning collection of urine.
strict aseptic condition. Prepare the skin over the
vein as for a surgical procedure. Clean with 70% Specimens for anaerobic culture
alcohol and apply povidone iodine. Allow about
Anaerobes can become nonviable even
a minute for the iodine to act. Do not touch this
within 30 minutes, when exposed to atmospheric
8
2003; 5(2):97
oxygen. Therefore samples collected without Blood for Serology

additives should reach the laboratory within this
period. Serum is most commonly used for antibody
tests. Collect about 5ml blood (more if several
Aspirated pus and biopsies are ideal for tests are required) using a dry syringe and transfer
anaerobic culture. Blood for anaerobic culture into a clean dry test tube without anticoagulants.
should be inoculated directly into appropriate Remove the needle before expelling blood into
media. Specimens collected using swabs and the tube. If serum should be transported or stored
sputum give unreliable results on anaerobic for a few days, use sterile tubes. To prevent serum
culture and should not be sent. from being chylous, collect blood prior to food
intake or only after 4 hours following food.
Specimens for fungal culture
For most antibody detection tests, blood can
Culture is most often done to diagnose be refrigerated for a day if delay is unavoidable.
subcutaneous, systemic and opportunistic fungal Blood collected for cold agglutination test should
infections. Biopsy of the affected site is the most not be refrigerated prior to serum separation as
reliable specimen. Swabs are not suitable. Do antibodies can attach to erythrocytes in the cold.
not refrigerate the samples, as some fungi are After separation, serum can be refrigerated
temperature sensitive. If there are discharging for a few days and frozen for a few months,
sinuses, collect and send any granules seen on without loss in antibodies.
the gauze dressing.
For functional assays like complement
CSF should be tested for diagnosing assay, serum should be sent immediately to the
Cryptococcal meningitis. This should be sent in laboratory (to be received within 30 minutes) as
sterile containers, clearly mentioning the complement components are heat labile.
suspected diagnosis.
Antigen detection tests
For suspected pulmonary fungal infections,
BAL may be sent. However, results should be Most of these are commercial tests.
interpreted carefully. Candida spp grown from Manufacturers instructions should be followed
sputum and even BAL are most likely from oral for specimen collection and transport. In general,
or oesophageal candidiasis. Similarly, collect the specimen from the site of infection. .
Aspergillus spp could be environmental
Elizabeth Mathai
contaminants. Therefore, always correlate culture
Professor, Department of Microbiology,
report with clinical features; send another sample
Christian Medical College, Vellore, Tamilnadu
- which should also yield the same fungus, and
request for a microscopy report of original Further reading
sample. In true infections the organism will be
seen almost always on microscopy of the sample. 1. Guidelines for collection, transport, processing,
analysis and reporting of cultures from specific
specimen sources. In: Koneman EW, Allen SD,
To diagnose P.carinii infection, coughed out
Janda WM, Schreckenberger PC, Winn WC
sputum is not suitable. Induced sputum after
(ed) Color atlas and text book of diagnostic
saline nebulisation can be used. Better still are microbiology, Lippincott, Philadelphia 1997;
BAL and lung biopsy. pp 121-170
9
2. Miller JM, Holms HJ. Specimen collection, 3. Myers and Koshis manual of diagnostic pro-
transport and storage In: Murray PR, Baron EJ, cedures in medical microbiology and immu-
Pfaller MA, Tenover FC, Yolken RH (ed) nology/serology, compiled by faculty of micro-
Manual of Clinical Microbiology 7th ed, Ameri- biology, Christian Medical College, Vellore,
can Soceity of Microbiology Press, Washing- 2001
ton DC 1999; pp 33-63
NEWS AND NOTES
USE OF IAP LOGO

1. The individual primary member of the IAP can use the Logo on his/her academy/professional
card and letterhead and cannot use the Logo on prescription pads and trade cards.
2. Every primary members has the privilege to use IAP Logo sticker on his personal vehicle,
however he/she cannot use it on commercial vehicles owned by them.
3. Primary member of any branch can use Logo for propagating the objectives of the IAP in the
Media / AIR/DD/Newsletter/Bulletin however local branch should be informed of this academic
activity.
4. The local branch/state branch/chapter/group/cell/center/committees cannot sale the Logo or use
it on the accessories for the purpose of the making funds for its activities. The propriety and the
power of use of Logo for sale or use by the trade lies with the Central IAP without voiding the
norms of the Academy.
5. It is local branch/state branch/chapter/group/cell/center/committees to print Logo on the
Letterheads and all the official correspondence by the office bearers should be done on this
letterhead.
6. The communication sent on IAP letterhead is presumed to be the voice of the IAP in unanimity
expressed by the Office Bearers of that concerned local branch/state branch/chapter/group/cell/
center/committees if any primary member wants to express his/her personal views may express
on the personal letterhead and views are purely personal and individual.
7. Avoid individual criticism on IAP letterhead even as an officer bearer of the particular local
branch/state branch/chapter/group/cell/center/committees, such members who are disregarding
this norm would invite disciplinary action by the Executive Board.
8. The Executive Board of IAP has the privilege and authority to warn any such member whose
writings are controversial, disrespecting and not conducive to the ideals of the IAP and direct
him not to indulge in a manner which would undermine the prestige of the IAP.
9. In spite of memorandum of warning if the member is not conducting properly, the Executive
Board has the right to temporarily suspend the member from the primary membership for a
period of one year and may be re-instate into the academy after reviewing his/her conduct by the
EBM. During the period of suspension such member cannot officially participate in the IAP
meeting or contest any from of IAP Elections.
10. It is the responsibility bestowed on Executive Board to be vigilant and alert on such activities of
the primary members, which are derogatory to the mission of the IAP.
10
2003; 5(2):99
LABORATORY MEDICINE
INTERPRETATION OF The interpretation of various investigations will

LABORATORY INVESTIGATIONS be dealt as follows
IN THE DIAGNOSIS OF Viral Infections
INTRAUTERINE INFECTIONS
1. Rubella: [German measles]
* Indrashekar Rao This human specific RNA virus is a member
Introduction of togavirus family causing mild self-limiting
infection in adults but has a devastating effect
Maternal infection acquired shortly before on fetus. Infection can occur at any time during
conception or during gestation can adversely pregnancy but early gestation infection is very
affect pregnancy outcome either by non destructive. Congenital rubella syndrome
specific effects of maternal illness or directly presents as cataract, IUGR, retinopathy,
through microbial invasion of fetus or neonate. microcephaly, meningoencephalitis, congenital
heart disease, splenomegaly, thrombocytopenic
The organisms causing intrauterine purpura. With maternal infection in first 12
infections can be classified as : weeks, the rate of foetal infection is 81%. As
Viral: Rubella, CMV, HSV, HIV, VZV, the gestational age advances the infection rate
Parvovirus , hepatitis, coxsackie drops. Risk for congenital defects is low if foetal
infection occurs beyond 20 weeks.
Protozoal : Toxoplasma, malaria
Spirochaetal : Treponema Prenatal diagnosis: It is mainly done by isolating
virus from amniotic fluid and by identification
Bacterial : Listeria monocytogenes
of rubella specific IgM by percutaneous
The mode of infection can be haematogenous umbilical blood sampling. These tests are
across placenta, ascending infection from limited by sensitivity and specificity.
infected cervix or due to contact between Postnatal diagnosis: Definitive diagnosis is by
fetus and infected genitalia during parturition. virus isolation in pharyngeal washings, CSF,
There is a possibility of intrauterine conjunctiva and lens at autopsy. Rubella virus
infection in an infant if there is a known RNA can be detected by PCR in clinical
exposure of mother to infectious agent or if specimens. Detection of rubella specific IgM in
the infant is small for gestational age or there neonatal or cord blood is also confirmatory. In
is failure to thrive. The other manifestations addition to the congenital defects, the persistent
are petechiae, hepatosplenomegaly, congenital rubella specific IgG with no decline as expected
malformations, thrombocytopenia and skin rash. from maternally acquired IgG points to the
diagnosis of congenital rubella syndrome. The
* Professor of Pediatrics interpretation of ELISA for rubella specific
Institute of child health, antibodies is as given in Table 1.
Niloufer hospital, Hyderabad [A.P.]
11
Table 1. Interpretation of ELISA for rubella specific antibodies

Age of infant Test result Interpretation
0 - 3 Months IgM + Intrauterine infection
3 - 12 Months IgM + Probably intrauterine infection
IgG + although widespread early post-
natal infection cannot be ruled out
[Rubella IgM/IgG Negative < 0.9 U/ ml
Equivocal 0.91 - 1.1 U/ml
Positive > 1.1 U/ ml]
Persistence of rubella specific haemagglutination inhibition titres beyond the period
expected from that of passively transferred maternal antibodies
2. Cytomegalovirus Isolation of virus or demonstration of CMV

DNA by PCR from urine or saliva at birth or
Infection by members of herpes virus within 1 - 2 weeks indicates definitive diagnosis.
family is characterised by typical cytopathology When detected after 2 weeks it might have been
of infected cells like cellular enlargement with perinatally acquired. Shell viral cultures are
intranuclear and cytoplasmic inclusions . The used for better isolation.
rate of intrauterine transmission from primary
The determination of CMV antibody titres
maternal infection is 30-40 %. Approximately
has got limited use. (Table 2)
18% develop significant disease. In mothers
with recurrent infection the fetus and newborn Table 2. Interpretation of CMV anti-
rarely show clinical symptoms. Risk of body titres
transmission in relation to gestational age is
uncertain although infection during early Antibody Result Interpretation
gestation carries a higher risk of fetal disease.
IgM + VE unreliable for
Symptomatic CMV presents either as an diagnosis; low
acute fulminant infection with multisystem sensitivity
involvement like petechiae, purpura, hepato
IgG + VE can be maternally
splenomegaly and jaundice. A second insidious
acquired; on
presentation is with microcephaly, IUGR,
followup positive in
chorioretinitis, periventricular calcifications,
infected infants
mental retardation, hearing deficits, motor
abnormalities, visual disturbances etc. IgG - VE In both mother
Prenatal diagnosis and fetus excludes
congenital infection
Amniocentesis and cordocentesis to detect
CMV DNA by PCR. CMV IgG/IgM antibodies
Postnatal diagnosis Negative : < 0. 91 U / ml
Infant presents with the characteristic Equivocal : 0.91-1.1 U / ml
symptoms. Positive : >1.1 U / ml ]
12
2003; 5(2):101
3. Herpes simplex virus Virus co-culture, p 24 antigen detection ,

HIV specific IgM / IgA all three have
There are 2 distinct types and type 2 is low sensitivity in 1st week of life .
an important cause of neonatal disease.
Intrapartum transmission is the most common Early diagnosis of HIV DNA is by
mode of transmission and is associated with PCR assay, positive predictive value in
active shedding from cervix and vagina . Intra neonates is 56% and older infants is
uterine infection is rare. Diagnosis is by high 83%.
degree of clinical suspicion . An infant presents Interpretation of PCR assay
with the disease as vesicles on 6 th 9 th day
over skin , eye and mucocutaneous membranes. Age Result Interpretation
One third of affected children present with
At birth 30 50 % + ve Intrauterine infection
encephalitis and another one third with
disseminated infection with seizures, shock and 7 days -ve at birth Intrapartum
DIC / hepatitis . but + ve transmission
at 7 days
IgM serology is of little use as it may not
reach diagnostic levels upto 3 weeks. False positive PCR : contamination with
maternal blood
Virus isolation from lesions of oro and
nasopharynx . Child exposed to HIV should be tested
at 1, 2 , 4 months by viral culture and
In encephalitis identification of viral DNA
PCR. If negative at 4 months there is a
in CSF by PCR , increased CSF protein
> 95 % assurance that infant is not infected.
levels and pleocytosis .
Exposed infant is considered negative of
4. Human immunodeficiency virus
there are
HIV is a cytopathic RNA retro virus - no physical finding of infection
which enters the host CD 4 cell and destroys
- virological tests are - ve
the host immune system. Inutero and
intrapartum transmission from infected mother - immunological tests - CD 4 counts
accounts for 90 % of paediatric cases. are normal
Transmission can occur throughout gestation - > 12 months of age and two or more
and HIV has been isloated from cord blood HIV antibody tests are negative .
and products of conception as early as 14 5. Varicella zoster virus
20 weeks of gestation .
It is a member of herpes virus family.
Diagnosis: In the early neonatal period is Intrauterine transmission occurs but prior to
difficult as HIV specific IgG antibody is peripartum period no obvious clinical
passively transferred. The median age of impairment is seen. Congenital varicella
clearance is 13 months. syndrome following maternal infection in first
Positive IgG in a child less than 18 trimester is 2 %
months indicates maternal infection but Prenatal diagnosis: By sonographic
cannot diagnose fetal infection . abnormalities like hypoplastic extremities,
13
clubfoot, flexed limbs, ventriculomegaly, rate in mothers with high viral loads.
porencephalic cyst, polyhydramnios, ascites, Coinfection with HIV increases transmission.
hydrops and calcification of lungs and
myocardium . Diagnosis
VZV specific IgM in fetal blood is Children born to HCV infected women
nonspecific screened at 6 12 months by PCR assay of
HCV RNA
VZV DNA detection by PCR in amniotic
fluid is specific Age Test result Interpretation
[HCV/RNA by PCR]
At birth: Virus isolation from vesicular fluid
on culture. Demonstration of four fold rise in 0-6 mo + ve can be due to
VZV antibody titre by fluorescent antibody to maternal antibody
membrane antigen.
6 -12 +ve Reliable of fetal
6. Parvo virus infection
These are small unenveloped viruses, most b. Hepatitis B
infectious strain is B19 whose overall rate of
transmission from an infected mother to fetus Intrauterine transmission is rare.
is 33%. Risk of fetal loss is 10 % . It has been Detection is by HBsAg specific IgG in serum
firmly linked to nonimmune fetal hydrops by
SPIROCHETAL INFECTIONS
affecting the haematopoietic cell lines. If acute
or recent parvovirus B 19 infection is Syphilis
confirmed in pregnant woman by positive
B19 specific IgM assay, serial ultrasonogram Syphilis can be transmitted to the infant
should be done for fetal hydrops or meconium regardless of duration of maternal disease but
peritonitis. more so in the first year of infection.
Transmission occurs more commonly after fourth
Prenatal diagnosis: Fetal blood and amniotic month of pregnancy. Liver is the primary site
fluid for parvovirus IgM specific antibody . of infection followed by secondary spread to
PCR to detect B 19 DNA . 100 % results skin, mucous membranes and bones.
when fetal blood is subjected to insitu Prenatal diagnosis: Amniocentesis and fetal
hybridisation . blood sampling to detect spirochaetes by:
Postnatal diagnosis: Serum IgM rises in 3
- dark field microscopy
days and falls by 2 3 months. Serum IgG
appears a few days after IgM disappears and - indirect immunoflourescent staining
lasts for years . - inoculation in rabbit
7. Hepatitis viruses Detection of DNA by PCR at 17 weeks of
a. Hepatitis C gestation is also diagnostic.
At birth: Dark field examination of
Single stranded RNA virus related to
mucocutaneous lesions.
flavivirus family. There is high transmission
14
2003; 5(2):103
If RPR and VDRL titres are fourfold Levels of maternally acquired IgG drops
greater than maternal titres, fetal infection at the rate of 50% per month, if not so
is very likely active fetal infection is suspected.
IgM fluorescent antibody is false positive In infants, less than 3 months of age
in 35 % due to interference by fetal IgM transformation of lymphocytes on exposure
directed against maternal IgG. This can to toxoplasma antigen is a sensitive test.
be minimised by separating both fractions.
2. Malaria
Congenital neurosyphilis is difficult to
diagnose. CSF mononuclear pleocytosis, It is an obligate intracellular protozoan of
increased protein, reactive CSF VDRL genus plasmodium. Neonatal infection has been
may indicate infection. recorded with all species. The placenta has been
involved in most women who acquire malaria
PROTOZOAL INFECTIONS during pregnancy. It is not clear whether
1. Toxoplasmosis transmission to infant is transplacental or from
direct contact with maternal blood during
An intracellular protozoan with 30-40 % parturition.
vertical transmission. Rate increases with
gestational age at which acute infection occurs Most infants have onset of symptoms by
and appears to correlate well with increasing 8 week of life with fever, anemia and
th
placental blood flow and is 90 % at term. The splenomegaly. About one third have jaundice.
severity of fetal disease is inversely
proportional to gestational age. Primary Diagnosis
neurological disease presents with intracranial Maternal history of febrile illness can be
calcifications, chorioretinitis and convulsions. elicited in most cases. The diagnosis of
The generalised disease presents with congenital malaria can be entertained in any
hepatosplenomegaly, lymphadenopathy infant who presents with fever, anemia,
,jaundice and anemia. hepatosplenomegaly and born to mother who
Prenatal diagnosis resided in an endemic area .The parasite can
be identified in cord blood and peripheral blood
Serial USG to detect hydrops, ventricular
by thick and thin smear.
dilatation, cerebral or hepatic calcification
and ascites. BACTERIAL INFECTIONS
Amniocentesis to isolate organisms by Listeria monocytogenes
inoculation in mice or by tissue culture.
It is a nonsporulating, -haemolytic gram
PCR analysis-B1 gene amplification of
+ ve organism. Transplacental transmission is
amniotic fluid has 97 .4 % sensitivity
believed to be the most significant mechanism
Postnatal diagnosis for acquiring early onset disease although
Isolation of toxoplasma in infant blood ingestion of aspirated amniotic fluid before
peaks in first week delivery is possible. Infected neonates present
with sepsis and pneumonia. They manifest
Detecting antigen / DNA by PCR in body with anorexia, lethargy, vomiting, respiratory
fluids is diagnostic. distress, apnea, cyanosis and petechial rash.
15
Diagnosis repeat IgM concentration shows a fall in

Diagnosis is prompted by maternal history case of maternal transfusion but increased in
of still birth and repeated abortions. Diagnosis active infection . Due to recent advances direct
is by culture of blood , urine and CSF. Gram serological and DNA analysis of fetoplacental
staining shows gram variable organisms which unit is possible and it can be used to determine
look like diphtheroids. whether infection has occurred or not. In rubella
Conclusion it facilitates continuation of pregnancy, while in
To conclude, the specific IgM concentration toxoplasmosis it guides the choice of
in serum may be increased due to leakage of antimicrobial, but has no role in evaluating HIV
maternal blood. Under these circumstances a as the proceedure itself can inoculate the fetus.
NEWS AND NOTES
5TH NATIONAL CONGRESS ON PEDIATRIC CRITICAL CARE

Annual conference of Critical Care subchapter of IAP is hosted by IAP, Surat branch, on October 10th,
11th & 12th, 2003 at Hotel Holiday Inn , Surat. Theme of the conference: Demystification of Critical Care.
CME: October 10th, 2003. Conference: October 11th & 12th, 2003 Pre-Conference workshop / course,
a) PALS - 8th & 9th October b) Basic Pediatric Critical Care Course - 8th & 9th October
c) Workshop A - Advanced Mechanical Ventilation + All about Equipments - 9th October
d) Workshop B - Peritoneal Dialysis + IV access + All about Equipments - 9th October
Registration Details:
A) Conference: Till 30.04.03 Till 30.06.03 Till 15.09.03 Spot
1. IAP Member 2000 2400 2800 3400
2. P. G. Student * 1600 2000 2400 2800
3. Non IAP Member 2200 2600 3000 3600
4. Associate Delegate 1250 1250 1500 1500
B)Workshop /Course Till 30.04.03 Till 30.06.03 Till 15.09.03
1 PALS 1200 1200 1200 No Spot
2 Basic Pediatric Critical
Care course 2000 2250 2500 No Spot
For PG Student * 1500 1750 2000 No Spot
3 Workshop A 500 650 800 No Spot
4 Workshop B 500 650 800 No Spot
Note:- 1. Conference registration includes CME & Banquet.
2. Except PALS, registration for the conference is a prerequisite for participating in the
workshops / basic course.
3. Limited participants to be taken for all the 4 workshops on 1st come 1st serve basis.
4. P.G. Students are required to attach certificate from the Head of the Department.
5. Registration not required for children below 12 years.
For children between age 5 & 12 years, lunch / dinner coupons available at reasonable rate.
Organizing secretary, Dr.Kamlesh H. Parekh, Amruta Hospital, Raj Complex , Near Vaishno Devi Temple,
Bhatar Road, Surat. 395001, Ph: 3240141, 3244979, 3237280; Fax: 0261-8313636,
E-Mail:[email protected]
16
2003; 5(2):105
LABORATORY MEDICINE
DIAGNOSTIC APPROACH TO THE presenting with rheumatologic symptoms will

CHILD WITH ARTHRITIS either not have an underlying rheumatologic
disease or will remain undiagnosed 1 . This
* Ramanan AV underscores the importance of keeping a wide
** Akikusa JD differential diagnosis in mind and of ensuring
follow-up until such a time as the diagnosis
INTRODUCTION
becomes clearer or symptoms resolve.
Children presenting with acute arthritis
Trauma as a cause of acute arthritis will
represent a relatively common problem for the
usually be obvious, however it is not uncommon
practicing general paediatrician. A large
for a traumatic event to bring to attention a joint
proportion will have underlying conditions that
that has actually been swollen for some time.
are self limiting in nature and require no more
Clues to the presence of an underlying more
than symptomatic treatment for a short period of
chronic condition are wasting of the muscles of
time. The challenge in their acute assessment is
the ipsilateral limb, joint contracture, and in the
to identify those with conditions that require more
lower limb (if present for long enough) leg length
than just symptomatic therapy. This requires a
discrepancy with the ipsilateral side being longer.
good knowledge of conditions commonly
Acute traumatic injury as a cause of joint swelling
associated with acute arthritis.
should be diagnosed with caution if the traumatic
A small proportion of children will go on to event was not immediately followed by difficulty
have chronic arthritis, the commonest cause of in ambulation or refusal to weight bear. Acute
which is Juvenile Idiopathic Arthritis (JIA). It is painful swelling of lower limb joints in response
important to understand that this is a diagnosis to minor injury in young boys may also be the
of exclusion and that mimics need to be first sign of an inherited bleeding disorder.
considered before making it. This article will
Septic joints are usually extremely painful
review the basic clinical approach to the child
and held in a fixed position. In the case of the
with acute and chronic arthritis, covering issues
hip, this is in flexion, abduction and external
of differential diagnosis and initial management.
rotation. The child will usually be febrile and on
The child with acute arthritis laboratory testing will have evidence of raised
inflammatory markers (Erythrocyte
Acute arthritis for practical purposes is any Sedimentation Rate/ C-reactive protein/
arthritis present for less than 6 weeks. The leukocyte count). It is important to note that acute
diagnostic approach to the child with acute arthritis involving the hip joint as an initial
arthritis is outlined in Fig 1. It is important to manifestation of JIA is extremely uncommon and
keep in mind that up to 61% of children such a presentation mandates consideration of
* Division of Paediatric Rheumatology more common differentials including septic
Hospital for Sick Children arthritis, slipped capital femoral epiphysis,
Toronto, Canada transient synovitis and Perthes disease. In the
17
Traumatic injury of intracapsular structure:

SWOLLEN JOINT
Uncommon in the very young child
Diagnose with caution if the acute event is not painful enough
to prevent activity immediately
History of injury? Yes
Acute Joint Bleed (esp. if young):
May be first manifestation of haemophillia in the young child.
No
Pain and swelling following minor trauma
Significant Pain and Fever? Yes Septic Arthritis:
Joint extremely irritable- often held fixed.
No
Reactive/ Post Infective:
Recent Usually a monoarthritis of a large joint.
Acute Diarrhoea? Yes If polyarticular and migratory consider Rheumatic fever
Viral illness? Typical viruses Parvoviruses/Rubella/EBV/Mumps
Tonsillitis? Typical gut agents: Salmonella/Shigella/Campylobacter
Onset 7-14 days after acute illness
No Associated conjunctivitis/sterile urethritis suggest Reiters
History of syndrome. Fever may be absent
Inflammatory bowel IBD associated arthritis:
disease or chronic bloody Yes Usually a monoarthritis of large joints
diarrhoea? Peripheral arthritis usually reflects activity of bowel disease
No
Vasculitis:
Rash?
Commonest Henoch-Schonlein purpura.
(Palpable or on extensor Yes
Often very painful. Typically resolves quickly even without
surfaces)
treatment
No
Serum Sickness:
Recent Drug ingestion? Yes
Often associated with an urticarial rash
No Malignancy (e.g. Leukaemia/ Neuroblastoma):
Bone Pain? May present with true joint swelling pain
Lymphadenopathy? Yes Often constitutional symptoms e.g. fever/lethargy/pallor
Hepatosplenomegaly?
Juvenile Idiopathic Arthritis:
No
Peak age 1-5yr
Pain often surprisingly little compared other causes
Present > 6 weeks?
May affect any joint and multiple joints
Morning irritability/stiffness? Yes
Rarely presents in the hips as a first manifestation
Gradual refusal to
Unusual Infective Organism:
participate in usual activities?
Consider TB particularly if monoarthritis and from endemic
No area
1) Symptomatic treatment with NSAIDs
No Clear Diagnosis Yes
2) Organize on-going review
Adapted with permission from the Arthritis guideline of the Royal Childrens Hospital, Melbourne, Australia.
(http://www.rch.org.au/clinicalguide/pages/joint_acute.php)
Fig 1: Algorithm for approach to child with acute arthritis.

18
2003; 5(2):107
Table 1: Differential diagnosis chronic Reactive arthritides are perhaps the most
arthritis in children* common cause of acute arthritis in children.
Juvenile idiopathic arthritis Common agents are viruses such as Parvovirus,
Connective tissue disorders Rubella and Epstein Barr virus. In endemic areas
- systemic lupus erythematosus (SLE) hepatitis B should be borne in mind. Many of the
- juvenile dermatomyositis (JDM) bacterial causes of gastroenteritis can be followed
- mixed connective tissue disease (MCTD) by acute arthritis, usually of the large joints and
- scleroderma typically quite painful and associated with
- systemic vasculitis elevated acute phase reactants on laboratory
Childhood sarcoidosis testing. In such instances it is important to look
Inflammatory bowel disease associated for joint sepsis, from which this form of arthritis
arthritis may be hard to distinguish, by arthrocentesis and
Other synovial conditions culture of synovial fluid. Streptococcal infection
- pigmented villonodular synovitis may give rise to subsequent Rheumatic Fever or
- foreign body synovitis (plant thorn Post Streptococcal Reactive arthritis2. Typically
synovitis) the arthritis in both of these conditions involves
- synovial hemangioma large joints, and in the case of rheumatic fever it
- synovial chondromatosis is migratory and very sensitive to non-steroidal
Infectious arthritis (including tuberculosis) anti-inflammatory drugs (NSAIDs). Indeed
Reactive arthritis failure to respond to NSAID therapy within 48-
Periodic fevers 72 hours places the diagnosis in question. It is
Metabolic diseases important to remember that between 30% and
- mucopolysaccharidoses 60% of patients with their first episode of acute
- diabetes rheumatic fever will not have a history of
- mucolipidoses symptomatic pharyngeal infection 3, 4. Post
- hemochromatosis Streptococcal reactive arthritis tends to be more
* some of the causes of acute arthritis listed persistent and not associated with other features
in the algorithm could also lead on to chronic of acute rheumatic fever.
arthritis
Both serum sickness5 (most often related to
drug ingestion) and vasculitis (the most common
latter two conditions the child will not appear being Henoch Scholein Purpura), may give rise
toxic and the acute phase reactants should be to a painful arthritis, typically of the large joints.
normal. Transient synovitis tends to be seen most Associated features of these conditions will
often in the 3-8 year age group, whereas Perthes usually be the clue to these diagnoses.
is more commonly seen in an older age group
(5-10yrs) and is more common in boys. Transient Perhaps the most worrying cause of acute
synovitis is typically quite painful and may cause arthritis in children is malignancy, of which
the child significant difficulties in ambulation. leukaemia and neuroblastoma are the
Perthes disease is classically described as a commonest. While both may cause true acute
painless limp. Slipped capital femoral epiphyses arthritis6, they more commonly cause bone pain,
tend to occur in the teenage age group, typically which may be localized around joints and which
older overweight boys, who present with pain and will frequently wake the child from sleep. The
limp. pain associated with these conditions may be
19
Table 2: ILAR classification criteria for juvenile idiopathic arthritis: Durban 1997
Systemic arthritis Polyarthritis (RF positive)

Definition: Arthritis with, or preceded by, daily Definition: Arthritis affecting 5 or more joints
fever of atleast 2 weeks duration that is docu- during the first 6 months of disease, associated
mented to be quotidian for at least 3 days and with positive RF tests on 2 occasions atleast 3
accompanied by one or more of the following: months apart.
1. Evanescent, non-fixed erythematous rash Exclusion:
2. Generalized lymph node enlargement a. Absence of positive tests for RF on two
3. Hepatomegaly or splenomegaly occasions atleast 3 months apart
4. Serositis b. Presence of systemic arthritis as defined
Exclusions: None listed above
Oligoarthritis Psoriatic arthritis
Definition: Arthritis affecting one to four Definition:
joints during the first 6 months of disease. 1. Arthritis and psoriasis or
Two subcategories are recognized 2. Arthritis and at least two of the following
1. Persistent oligoarthritis: affects no more a) Dactylitis
than four joints throughout the disease b) Nail pitting or onycholysis
course c) Family history of psoriasis confirmed by
2. Extended oligoarthritis: affects a cumulative dermatologist in at least one first degree
total of five joints or more after the first 6 relative.
months of disease Exclusions:
Exclusions: 1. Presence of rheumatoid factor
a. Family history of psoriasis confirmed by a 2. Presence of systemic arthritis as defined
dermatologist in atleast one first or second above
degree relative Enthesitis related arthritis
b. Family history consistent with medically Definition: Arthritis and enthesitis, or arthritis
confirmed HLA B-27 associated disease in or enthesitis with atleast two of the following:
atleast one first or second degree relative 1. Sacroiliac joint tenderness and/or inflamma
c. Positive RF test tory spinal pain
d. HLA B-27 positive male with onset of 2. Presence of HLA-B27
arthritis after 8 years of age 3. Family history in atleast one first or second
e. Presence of systemic arthritis as defined degree relative of medically confirmed
above HLA-B27 associated disease
Polyarthritis (RF negative) 4. Anterior uveitis that is usually associated
Definition: Arthritis affecting 5 or more joints with pain, redness or photophobia
during the first 6 months of disease, associated 5. Onset of arthritis in a boy after the age of 8
with negative RF tests on 2 occasions atleast 3 years
months apart. Exclusions:
Exclusions: a. Psoriasis confirmed by a dermatologist in
a. Presence of RF atleast one first or second degree relative
b. Presence of systemic arthritis as defined b. Presence of systemic arthritis as defined
above above
20
2003; 5(2):109
Other arthritis Table 3: Standard Anti-inflammatory

Definition: Children with arthritis of unknown doses of NSAID
cause that persists for atleast 6 weeks but that Naproxen 15-20mg/kg/d in 2 divided doses
either
Ibuprofen 30-40mg/kg/d in 3 divided doses
1. Does not fulfill criteria for any of the
categories, or Indomethacin 2-3mg/kg/d in 3 divided doses
2. Fulfills criteria for more than one of the
patients and 65% of JDM patients will have
other categories
arthritis/arthralgia during the course of their
Exclusions:
illness7. A thorough clinical examination looking
Patients who meet criteria for other categories
for other features which might suggest these
extreme and may result in the child not weight diagnoses is essential. Such features include
bearing. Under these circumstances it is crucial Gottrons papules, heliotrope rash, proximal
that the child undergoes a thorough physical muscle weakness, mucocutaneous changes,
examination of the lymphoreticular system and alopecia and evidence of serositis.
at the very least a complete blood count taken.
Classic early onset childhood sarcoidosis is
Other investigations which may be required in
characterized by a triad of arthritis, uveitis and
order to ensure that a malignancy is not being
rash. The arthritis of childhood sarcoidosis is
missed are a bone marrow aspirate, urinary
characterized by boggy tenosynovitis with
catecholamines and CT or Ultrasound scan of the
relatively painless effusions and good range of
abdomen.
movement8.
Clearly some of the conditions associated
Arthritis is the most common extra-intestinal
with acute arthritis discussed above require
manifestation of inflammatory bowel disease.
specific therapy, but a great many require
There are two patterns of joint involvement:
symptomatic treatment only. If there is evidence
peripheral and, less commonly, sacroiliac arthritis
of true synovitis or joint effusion such treatment
(spondyloarthropathy). The peripheral arthritis
should be with NSAIDs at appropriate doses
usually reflects the activity of the underlying
(Table 3). With this therapy the majority of these
bowel disease, while, the sacroiliac arthritis tends
conditions will settle within 6 weeks. If they do
to follow an independent course9. Uncommonly,
not then consideration must be given to the causes
the arthritis may precede the bowel
of chronic arthritis in children
manifestations. In this situation the diagnosis is
The child with chronic arthritis suggested by the presence of abdominal
symptoms, occult blood in stools, low
Chronic arthritis is defined as the persistence haemoglobin, thrombocytosis, a low serum
of arthritis for 6 weeks or more. Common causes albumin and a positive pANCA (anti-neutrophil
of chronic arthritis are shown in Table 1. Of all cytoplasmic antibody), the latter found in 60-80%
the causes of chronic arthritis in children JIA is of cases.
the most common.
The differential diagnosis of chronic
Most connective tissue disorders including monoarthritis includes tuberculosis, in endemic
lupus, juvenile dermatomyositis (JDM) and areas, and local disorders of synovium. An
scleroderma may have arthritis as one example of the latter is pigmented villonodular
manifestation. Approximately 90% of lupus synovitis, a rare condition characterized by
21
recurrent painless effusions which, on aspiration, References

are heavily blood-stained. Both T 1 and T 2- 1. Rosenberg AM. Analysis of a pediatric rheu-
weighted MRI studies reveal hypertrophic matology clinic population. J Rheumatol 1990;
synovium, with very low signal density as a result 17:827-830.
of hemosiderin deposition 10 . Synovial 2. Jansen TL, Janssen M, van Riel PL. Grand
hemangioma, most commonly seen in the knee, rounds in rheumatology: acute rheumatic fe-
usually presents as an intermittent hemarthrosis. ver or post-streptococcal reactive arthritis: a
clinical problem revisited. Br J Rheumatol
The diagnosis of JIA is a clinical one. The 1998; 37:335-340.
use of investigations is primarily to exclude 3. Veasy LG, Wiedmeier SE, Orsmond GS, et al.
differential diagnoses and, once the diagnosis is Resurgence of acute rheumatic fever in the in-
made, to aid in classification and prognostication. termountain area of the United States. N Engl J
Perhaps the one exception is a slit-lamp Med 1987; 316:421-427.
examination of the eye, which can be very helpful 4. Lee LH, Ayoub E, Pichichero ME. Fewer
in the diagnostic work-up of a child with arthritis. symptoms occur in same-serotype recurrent
A significant proportion of children with JIA streptococcal tonsillopharyngitis. Arch Otol
develop clinically silent but potentially blinding aryngol Head Neck Surg 2000; 126:1359-1362.
chronic uveitis. On average, uveitis is seen in 5. Kunnamo I, Kallio P, Pelkonen P, Viander M.
15-20% of patients with pauciarticular and 5 % Serum-sickness-like disease is a common cause
of patients with polyarticular JIA11. The presence of acute arthritis in children. Acta Paediatr
of uveitis in a child presenting with arthritis Scand 1986; 75:964-969.
narrows the differential essentially to JIA and 6. Tuten HR, Gabos PG, Kumar SJ, Harter GD.
sarcoidosis, of which the former is overwhel The limping child: a manifestation of acute leu-
kemia. J Pediatr Orthop 1998; 18:625-629.
mingly more common. The Inter national League
Against Rheumatism (ILAR) has classified JIA 7. Tse S, Lubelsky S, Gordon M, et al. The arthri-
into seven categories with inclusion and exclusion tis of inflammatory childhood myositis syn-
dromes. J Rheumatol 2001; 28:192-197.
criteria for each type (Table 2)12. It is important
to remember that these criteria were proposed 8. Shetty AK, Gedalia A. Sarcoidosis in children.
Curr Probl Pediatr 2000; 30:149-176.
mainly to classify patients for research and
prognostic purposes, but as with many such 9. Passo MH, Fitzgerald JF, Brandt KD. Arthritis
associated with inflammatory bowel disease in
criteria, they also serve as a diagnostic tool.
children. Relationship of joint disease to activ-
Initial management of chronic arthritis: As ity and severity of bowel lesion. Dig Dis Sci
with acute arthritis the initial management of a 1986; 31:492-497.
child presenting with chronic arthritis revolves 10. Bravo SM, Winalski CS, Weissman BN. Pig-
around symptomatic relief, which in most mented villonodular synovitis. Radiol Clin
instances will be an NSAID at anti-inflammatory North Am 1996; 34:311-326.
doses (Table 3). Ongoing management will 11. Cassidy JT, Petty, R.E. Juvenile rheumatoid ar-
depend on the underlying diagnosis. Whatever thritis. In: Cassidy JT, Petty RE, editors: Text-
book of Pediatric Rheumatology 2000:pp218-
the cause, it is important to remember that patient/
321.
parent education and physiotherapy are integral
12. Petty RE, Southwood TR, Baum J, et al. Revi-
to the successful management of many of the
sion of the proposed classification criteria for
conditions which result in chronic arthritis in
juvenile idiopathic arthritis: Durban, 1997. J
children. Rheumatol 1998; 25:1991-1994.
22
2003; 5(2):111
LABORATORY MEDICINE
RAPID DIAGNOSTIC TESTS An ideal diagnostic tool needs to fulfill certain

BENEFITS AND PITFALLS requirements:
* Saranya N It should be easy to perform
The last decade has indeed been the decade Should not require expensive equipment
of diagnostics. To keep pace with the emergence Should not require electricity if possible, so
and re-emergence of several infections, it can be applied to field situations too
manufacturers have not left any stone unturned
Results obtained should be repeatable
in improving the quality of the available kits. In
addition, the need for faster and equally accurate, It should be highly sensitive and specific
reliable, sensitive and specific rapid kits has been It should aid in the diagnosis
felt more urgently. These rapid testing kits /
methods can be broadly divided into two In the pediatric age group in India, the
categories those that involve single step and infections commonly encountered are malaria,
depend on agglutination, flow-through and lateral typhoid, dengue, leptospirosis, tuberculosis and
flow etc. which take a few minutes and those that Hepatitis A. Several other viral and bacterial
involve multiple steps like ELISA, Passive infections are encountered too, though much less
Haemagglutination (PHA) and Molecular tools, frequently. The rapid tools available for these
which may take upto a few hours. infections will be dealt with, in reference to their
benefits and pitfalls.
Rapid testing devices
Typhoid
A major problem worldwide and is
Single step Multiple steps particularly rampant in the developing nations.
Early detection is vital for its control. With the
more recent blood culture techniques available,
it is possible to isolate, identify and give a
Agglutination ELISA Molecular complete blood culture report within four days.
Chromatography PHA PCR Some of the commercially available blood culture
WB DNA etc. media have an incorporated resin, which absorbs
(western the antibiotic that the patient might have been
blot) treated with. Hence the drawback of false
negatives in blood culture as a result of antibiotic
Flow-through Lateral flow usage in the patient has been removed. Table I
lists the merits and demerits of the available
* Director, diagnostic tests.
Lister Laboratory and Research Centre
Chennai 34.
23
Table 1. Properties of diagnostic tests used in typhoid fever

Properties Culture (Ag) PCR (Ag) Widal (Ab) Rapid tests (Ab)
Quality Gold standard Promising Not useful (single) Not useful
(single)
Utility First week Early Second week 4 7 days
Sensitivity 45 70% 72% 36% 95%
Specificity 100% 100% Non specific *High NPV 96.1%
(dead bacilli)
Result 4 days 1 2 days Overnight 1 hour IgG
3 hours IgM
Cost Moderately Expensive Inexpensive Moderately
expensive expensive
* Negative Predictive Value
Rapid tests for typhoid are based either on of ratio between blood and media and so on, a
the principles of EIA (IgM and IgG antibody), definitive diagnosis of typhoid can be reached
immuno-chromatography (antigen detection) or within a day.
agglutination. A positive test result even if
antigen is present, needs ideally to be Malaria
confirmed with a more specific test, while a
negative result does not preclude the presence Clinical or syndromic diagnosis is often used
of infection. When an IgG test alone is positive, to identify and treat malaria in remote areas where
a positive blood culture done a few days later diagnostic facilities are not available. In addition,
will resolve the issue of re-infection or relapse. symptoms of malaria are often non-specific and
Detection of S. typhi infection using labelled result in mis-diagnosis. Four species of malaria
probes is 99% specific but a minimum of 500 that commonly infect man are the Plasmodium
organisms per ml is required while with PCR it vivax, falciparum, malariae and ovale. Of these
is 10/ml and with nested PCR 1-5 organisms per the P.falciparum causes the most fulminant forms
ml is sufficient to detect an infection1. of infection while P.vivax is the most frequently
seen in India. Diagnostic techniques include the
Widal test has outlived its usefulness and time honoured blood films both thick and thin,
more often than not a very ambiguous report is the quantitative buffy coat technique (QBC) and
obtained. The test is most often ordered as the several immuno-chromatographic tests based on
initial and often the only diagnostic tool in the Dipstick format. These are based on the
patients with suspected typhoid and when the principle of detection of plasmodial histidine rich
result is negative, a diagnosis of typhoid is no protein-2 (HRP-2) or parasite specific lactate
longer considered. This test gives numerous false dehydrogenase (pLDH) that is present in
positives that have led to unnecessary and P.falciparum infections. These tests are now
ineffective medication. being applied for other forms of malaria but the
results have not been encouraging2. Table II
Blood culture remains the gold standard and compares the commonly used tests.
when done with observation of all necessary
precautions such as time of sampling, aseptic Positive results obtained in the IC test need
procedures, volume of blood drawn, maintenance to be confirmed by a more specific test. Rapid
24
2003; 5(2):113
Table 2. Properties of tests used in diagnosis of malaria

Properties Smear QBC IC (Dipstick)
Usefulness Gold standard Extremely sensitive Falciparum
Principle Routine staining Flourescein stain Immuno Chromatography (IC)
Parasitic forms All All Falciparum Antigen
Sensitivity Can be missed 100% 100%
Specificity 100% False positives False positives
tests are useful for screening and confirmation Dengue

especially when,
This infection caused by a member of the
a) there are very few forms or Flavivirus family is transmitted to humans
b) if an inexperienced person is doing the through the bite of the Aedes mosquito. Antibody
testing2. appears within 3-5 days and is detectable for
around 90 days3. There are several methods used
Other recent field players are Enzyme
for the diagnosis of Dengue. PCR is used to detect
immuno-assay (EIA) and Multiplex Polymerase
the presence of the viral nucleic acid but is fairly
Chain reaction (PCR). EIA has been developed
expensive. Serological detection of Dengue
and evaluated, including tests for Pf HRP-2
infections are based on the principles of
antigen. However they only detect falciparum
haemagglutination inhibition, ELISA (IgM, IgG
malaria hence their usefulness is limited. They
and IgM and G combined) and the more rapid
equal microscopy in sensitivity, but are
tests being the Dipstick enzyme immuno-assay
impractical and expensive to use in the field. If
(IgM or IgM and G) and immuno-
an EIA test that was able to detect all forms of
chromatography to detect IgG alone or IgM and
malaria was available, that would perhaps be
IgG combined. Paired serum sampling is ideal
more useful.
to make a diagnosis of dengue and
A multiplex PCR has been developed for differentiate between primary and secondary
all four species using the target 18S single infections. Certain disadvantages of any IgM
stranded rRNA and serum sporozoite stage DNA test for dengue are that3
sequences. The Royal Tropical Institute
1) IgM may be undetectable for upto 5 days
Amsterdam has developed a qualitative Nucleic
and so a sample drawn earlier than this
Acid Sequence Based Assay (NASBA) method
period may give a false negative result. A
for detection and semi-quantification of as few
repeat sample drawn a week later will help
as 50 parasites per milliliter of blood, which is
resolve this issue.
many times more sensitive than microscopy.
However they are expensive to make, difficult 2) False positives in other flavi-virus infections
to run and need sophisticated equipment.
3) False positives as a result of stimulation by
Rapid tests can be used as supplemental non-flavi viruses.
tests but can never replace microscopy in the 4) A positive result does not indicate an active
diagnosis of malaria. infection
25
All the serological assays can be completed problems, it is the remaining 10%-15% of patients
within a working day with the microtitre ELISA in whom early and rapid diagnosis is even more
taking about three hours and the immuno- imperative. Like in malaria, symptoms are non-
chromatographic tests about 15 minutes. Studies specific and can mimic any viral infection hence
done at Singapore have found that the microtitre this infection is often under-reported.
format capture ELISA for detection of IgM and
IgG when used together was superior to the use Rapid diagnostic tests for leptospirosis can be
of IgM or IgG alone, and showed good divided into two groups:
correlation (sensitivity 99%, specificity 96%)4 Those based on the presence of antigen
when compared to the haemagglutination
inhibition assay which is considered to be the a) Dark field microscopy (DFM) and special
ideal serological test. stains
In summary, there is no single perfect b) Molecular techniques like PCR, RAPD,

diagnostic test for dengue. While viral isolation hybridisation methods and PGE
gives the best chance of a definitive diagnosis, it
These are ideal tools for the early diagnosis
takes time and cannot be performed in all
of leptospirosis. Molecular techniques are
laboratories. Clinical judgement therefore is
extremely sensitive, very expensive and are not
very important particularly in dengue and
available for routine use. Microscopy has been a
simple laboratory tests like a platelet count or
severely under-utilised tool and while the risk of
haematocrit should be used to institute the
false positives does exist, if done by a trained
appropriate supportive treatment without any loss
microscopist it can be extremely specific6.
of vital time3.
Those based on antibody presence
Leptospirosis
a) IgM detection assays like ELISA and Dipstick
A geographically widespread spirochetal
infection caused by members of the genus b) Agglutination tests like the PSAT, MAT, IHA
Leptospira, this infection has assumed endemic
proportions in certain states namely Andamans, c)Immuno-blotting assays like the Dot Blot assay
Gujarat, Tamilnadu and Kerala. More than 230
A commercially available IgM ELISA kit
serovars have already been identified. The
has been evaluated against the gold standard of
organism is shed in the urine of the reservoir host
the MAT and found to have a sensitivity of
into the environment and man acquires the
100% 7. The IgM PK ELISA with 89.9%
infection when the organism enters through
sensitivity and 97.4% specificity and the
cracks in the skin and mucous membranes.
Leptotest-S with an 89.9% sensitivity and 94.8%
Anicteric presentations account for 90% of
specificity have been found to be ideal for early
human leptospirosis. Icteric and anicteric cases
diagnosis in most laboratories according to
follow a biphasic course. Weils syndrome can
another study8.
be caused by any serovar in its severe form. All
forms of leptospirosis begin the same way and at Pitfalls of antibody detection in leptospirosis:
the start of infection it is not possible to predict
the outcome5. While almost 85% to 90 % of a) Paired sampling done 10-14 days apart is a
infections clear spontaneously with no residual must to demonstrate a rise or fall in titre.
26
2003; 5(2):115
b) A single positive titre is of no significance for the diagnosis of TB. Cultures are 81%
as raised IgM antibody levels are detectable even sensitive and 98.5% specific for active disease.
a year after infection. However reporting takes anywhere between 10
days with some of the newer techniques to three
c) Prior treatment with antibiotics may delay, to four weeks with the more conventional
blunt or supress antibody production. methods, and hence when an immediate diagnosis
IgM ELISA tests are able to detect antibody is required, culture cannot be used. Rapid
presence on an average five to seven days after indication of drug resistance is also possible by
onset of an infection, however while an initial using MTB specific mycobacteriophages to
sample might be negative, a second sample taken reflect the presence of viable tubercle bacilli in
a week later might show a significant rise in titre. the presence or absence of rifampicin9. The need
A practical problem that clinicians face is for early, rapid diagnosis is essential for prompt
accessibility of the patient after the initial visit. institution of treatment.
Hence the search continues for a test that can give Rapid tests for TB can be divided into two main
a conclusive diagnosis with a single sample. groups:
The MAT test has until recently been Direct
considered the cornerstone for leptospirosis
diagnosis, however, the focus of researchers the a) Sputum smear microscopy
world over is now shifting towards antigen based
molecular methods of detection. A simple, b) Continuous automated mycobacterial liquid
inexpensive, molecular tool that does not require cultures (CAMLiC)
skilled personnel and that can be used in any c) Molecular techniques
resource poor setting would be ideal. Ironically
enough, most of diagnostic research in the field Indirect
of leptospirosis has been done by the western
a) Line immuno-chromatographic serological
world, where leptospirosis is not as large a
assays (LISA)10
problem as in tropical countries where it is
endemic in some. b) ELISA
The MAT test is an excellent The best method for diagnosing
epidemiological and research tool but is not pulmonary TB is the sputum smear. PCR is
suitable as a rapid screening or diagnostic indicated in smear positive individuals who have
test5. not had treatment for longer than seven days.
While it takes 10000 organisms to produce a
Tuberculosis
positive smear, even a few are sufficient to give
Pulmonary TB is among the foremost killer a positive PCR report11. However, the PCR does
diseases in India. According to estimates of the not differentiate between dead and live bacilli and
WHO, 90 million new active cases have been hence a PCR report should be interpreted with
identified worldwide of whom a third have caution. Rapid serological tests for the diagnosis
already died. With AIDS having assumed of tuberculosis have sensitivities between 13-92%
gigantic proportions, the situations can only and specificities between 66-100%12. A negative
worsen. Sputum culture remains the gold standard rapid diagnostic test in a patient with low clinical
27
suspicion and a positive AFB smear will be to prevent vertical transmission

helpful to eliminate the presence of active
b People who may have exposed themselves
infection. Conversely a positive rapid test, when
to an occupational risk (medical and nursing
the level of suspicion is moderate with a positive
staff)
smear will clinch the issue12.
c Screening before providing medical
Hepatitis A attention.
Commonly referred to as infectious A negative rapid test result in an area of low
hepatitis, this is caused by a picorna virus. This prevalence does not pose a problem. Any patient
spreads almost exclusively through faeco-oral with a positive test result on a rapid test needs to
contact, from person to person through have it confirmed by a western blot.
contaminated food or water. IgM Antibody to the
virus is detectable five days after exposure and A HIV antibody positive report in a newborn
remains detectable for 3-6 months. During the needs to be interpreted with caution. In the
convalescent phase individuals produce IgG newborns blood, maternal IgG is present as,
antibody, which usually remains as a lifelong a)during pregnancy, there is a passive transfer
marker. Diagnosis is usually made on the basis of maternal IgG antibody to the newborn, b) there
of clinical symptoms and signs and the need is admixture of small amounts of maternal and
for testing more for a confirmation of foetal blood and c) there are placental leaks. This
diagnosis and identification in those situations antibody remains detectable in the blood of the
where the ink-filler principle operates. The newborn even up to one year, hence these results
affected individual has two or three infections should be correlated with the maternal HIV status
simultaneously and the clinical picture is totally when possible. If a conclusive diagnosis of HIV
confusing. Serological diagnosis is by detection is required in the newborn, a qualitative
of IgM antibody or total antibody to Hepatitis A polymerase chain reaction is ideal. With a
virus. Several commercially available ELISA kits qualitative PCR, the sample can be reported
are used and usually take about three hours to conclusively as either being negative or positive
perform. These tests are extremely reliable and for copies of the virus.
offer good degrees of sensitivity and specificity13. In adults, rapid testing of saliva and urine for HIV
HIV antibody has become common over the counter
tests in some of the developed countries. The
An emerging area of great concern is that saliva tests have a sensitivity of 99.5% and those
of vertical transmission of HIV. This is possibly of urine are 98.7% sensitive and 99.1% specific.
one situation where rapid tests are most indicated. However at a HIV point of care testing
Rapid tests for HIV are based on different workshop in Canada in March 9916, it was
principles like particle agglutination, membrane underlined that since 2/3rd of people who test
immuno-concentration, immunochromatography positive on rapid testing devices subsequently
and ELISA. The sensitivity of these devices is tested negative for HIV by the western blot, rapid
around 97-100% with specificity of 96%14. Rapid testing devices need to be used only in certain
tests are indicated in certain situations15: specific situations as the benefits of easy testing
are far outweighed by the enormous anxiety the
a Pregnant women whose HIV status is individual undergoes before his/her HIV status
unknown, during the time of delivery so as is confirmed.
28
2003; 5(2):117
Rapid testing devices are a great innovation, tection of IgM antibodies in the diagnosis of
do save considerable time and can be used in human leptospiral infection. J clini microbiol,
certain places where sophisticated equipment or 1997; 35 (8) 19381942.
trained personnel are not available. However 8. Ribeiro M A, Brandao A P, Romero E C. Bra-
wherever and whenever indicated they need zilian J, Evaluation of diagnostic tests for hu-
to be confirmed before the report is disclosed man leptospirosis, Medical Biological Re-
to the patient. They need to be interpreted after search, June 1996; 29 (6): 773777.
taking into account the prevalence of that 9. Trollip A, Albert H, Mole R, Hatch S, Blumberg
particular infection/disease in the population L. Biotec Laboratories Ltd, South Africa and
under study. All laboratory tests are only guides UK and South African Institute for Medical Re-
to a diagnosis and should be clinically correlated search, Johannesburg, South Africa, Rapid in-
in all situations. dication of MDR-TB from automated liquid
culture systems using FAST plaque TB-RIFTM
References test.
1. Abdul Haque, Jaabaz Ahmed, Javed A Qureshi. 10. Evaluation of the Rapid Line Immuno-chro-
Early detection of typhoid by Polymerase Chain matographic serological assay for presumptive
Reaction. Ann Saudi Med 1999; 19 (4) : 337 detection of M.TB infection. Tuberculosis
340. project 2 4 96, J N International Ltd., My-
cobacterium Tuberculosis diagnostics and vac-
2. Malaria Laboratory Diagnosis R P H Labo- cine projects (1996 2002).
ratory Medicine; 1998 2000
11. Rapid Diagnostic Tests for tuberculosis:
3. Guidelines for the diagnosis and management Progress but no gold standard An editorial
of dengue for health care staff in N.Queensland. Am J. Resp. Crit. Care Med, 1997;155: pp 1497
Published by the Peninsular and Torres Strait 98.
region, the northern region and the Mackay
region of Queensland health in 1994. Refor- 12. Dr.V.H. Balasangameshwara, Rapid diagnos-
matted by Keyan Daniell. tic tests for tuberculosis, Pre-congress work-
shop on rapid diagnostic test in clinical micro-
4. Sang CT, Cuzzubbo A J, Devine P L. Evalua- biology 6th Nov 1998, IAMM, Manipal.
tion of a commercial capture enzyme linked
immuno-sorbent assay for detection of immu- 13. Viral Hepatitisan epidemic in the making.
noglobulin M & G antibodies produced during Monograph provided by American Diagnostic
dengue infection Clinical Diagnostic Lab Im- Health Foundation in cooperation with Ameri-
munology 1998, January 5 (1) 7 10. can Liver Foundation.
5. Faine S, Adler B, Bolin C, Perolat P. Leptospira 14. Bernard M, Brasson MD. Rapid tests for HIV
and Leptospirosis. Chapter 12, Second edition, Antibody, AIDS Review 2000 (in press).
MediSci , Melbourne; Sept 1999. 15. Michele E. Roland, Richard Fine, Paul A
6. Saranya Narayan, Srinivasan P, Padmasree Volberding, Indication for the use of HIV An-
Ramesh. Leptospirosis - An emerging transfu- tibody tests - April 1998 AIDS Knowledge
sion transmissible infection. Paper submitted Base.
for presentation 16. Concerns about rapid HIV screening at the
7. Winslow W E, Merry D J, Pirc M L, Devine P point of care. HIV Point of Care Testing
L. Evaluation of a commercial ELISA for de- workshop held by Health Canada March 1999.
29
LABORATORY MEDICINE
NEONATAL METABOLIC with untreatable disorders, it is important to

SCREENING establish the diagnosis in the index case in order
to allow prenatal diagnosis in subsequent
* Nair PMC pregnancies. Hence it is imperative that practicing
paediatricians and neonatologists be familiar with
Every parent wants to bring a healthy baby
the clinical presentation and approach to these
into the world. The advances in medical sciences
disorders.
has led to a reduction in infectious diseases, so
that genetic and metabolic disorders are Clinical recognition of metabolic disease in the
remaining as a major group of disorders neonatal period
responsible for morbidity and mortality,
especially in neonates. Newborn screening An important key to diagnosis is a high index
program identifies biochemical or other inherited of suspicion. The signs and symptoms are quite
conditions that may produce mental retardation, nonspecific and subtle in onset or can be stormy.
other disabilities and/or death. There are 2 types Consider the possibility of a metabolic disease
neonatal screening for high risk neonate, mass in any infant with non-specific symptoms that
newborn screening. The latter is decided by are not explicable by another cause.
discord prevalence in a particlar area and by the
Look for history of 1) consanguinity (the
availabilty of laborotary facility reservoiers. The
majority of metabolic diseases presenting in the
most commonly accepted diseases needing
newborn period are autosomal recessive),
neonatal screening are1 Hypothyroidism, Phenyl
2) positive family history of similar disease /
ketonuria, Maple syrup urine disease, Biotinidase
unexplained neonatal deaths in siblings, mental
deficiency, Galactosemia, Tyrosinemia,
retardation, developmental delay or intolerance
Histidinemia, G6PD deficiency, Sickle cell
to certain foods. 3) Most often it is a full term
disease, Cystic fibrosis, Congenital adrenal
baby born after an uneventful perinatal period.
hyperplasia, Homocystinuria
Prior to delivery, the fetus is protected from
Around 500 metabolic diseases are known any ill-effects of a metabolic disease by virtue of
today. Approximately 24 children per one lakh the function of the placenta in providing fuel and
births have a disease involving amino acids, filtering toxic metabolites. It takes some time for
organic acids, primary lactic acidosis, the toxins to build up, so that for the first few
galactosemia or a urea cycle disease2. Recent days after birth, the baby is usually asymptomatic.
advances in the diagnosis and treatment of inborn 4) Acute onset with progressive course- starting
errors of metabolism have improved the with some subtle symptoms like lethargy, poor
prognosis for many of these conditions3,4,5,6. Even feeding, vomiting, hiccoughs, respiratory
distress, apnea, and jaundice that soon progress
* Consultant Neonatologist,
to coma, seizures, multi-system failure and death.
Child Health Department,
Sultan Qaboos University Hospital, 5) Not to forget some metabolic diseases like
Muscat, Sultanate of Oman. Long Chain Hydroxy Acyl CoA dehydrogenase
30
2003; 5(2):119
Table I.Unusual odour or smell of urine Encephalopathy, seizures and apnea without a
Odour Disorder symptom-free interval is typical of
Musty or mousy odour Phenylketoneuria a) Primary lactic acidosis
Boiled cabbage smell Tyrosinemia b) Non-ketotic hyperglycinemia (NKH)
Smell like burnt sugar Maple syrup urine
c) Sulphite oxidase deficiency (SOD)
(Maple syrup) disease
Sweaty feet smell Isovaleric acidemia d) Pyridoxine dependency
Glutaric acidemia If associated with profound hypotonia,
(TypeII) dysmorphism and / or congenital anomalies,
Cat urine smell Multiple Carboxylase myopathy think of a) Peroxisomal disorders b)
deficiencies Mitochondrial disease
Rotten fish odor Trimethylaminuria
3) Storage type: Hydrops, jaundice,
hepatosplenomegaly and dysmorphism may be
deficiency (LCHAD) in the fetus affecting the
present. eg: Mucolipidoses, Niemann Pick
mother with Acute fatty liver of pregnancy.
disease
Physical examination may yield nonspecific Cardiac disease: Cardiac failure and
findings like hypotonia / hypertonia, seizures, cardiomyopathy, can occur in association with
jaundice, hepatomegaly, cardiomyopathy, mitochondrial, lysosomal or fatty acid oxidation
dysmorphism or coarse facial features, disorders or Pompes disease (GSDII).
abnormalities of the skin, hair, eyes, joints,
unusual urine color, unusual odour of sweat or Liver dysfunction: Persistent hyperbiliru
urine (Table 1). Acute symptoms maybe binemia (conjugated +/- unconjugated) may be
indistinguishable from those of sepsis, indicative of a metabolic disease, in particular,
cardiorespiratory failure or CNS disease. galactosemia but also hypothyroidism,
Neutropenia, thrombocytopenia and sepsis tyrosinemia, alpha-1-antitrypsin deficiency and
particularly with E.coli may be present. Chronic others8.
symptoms are failure to thrive, developmental Dysmorphism: Metabolic diseases
delay or neurological defects. associated with dysmorphic features include
peroxisomal disorders (Zellweger syndrome),
Patterns of presentation of metabolic disease
disturbances of energy metabolism(Pyruvate
in the neonate and clue to diagnosis
dehydrogenase deficiency) defects in cholesterol
Three types of presentation7,8 biosynthesis (Smith-Lemli-Optiz syndrome,
CDG-Carbohydrate deficient glycoprotein
1) Intoxication type with a window period and syndromes) and storage disorders8.
progressive encephalopathy. Neurological
Fetal hydrops: A number of metabolic
presentation with a symptom-free interval,
diseases can cause fetal hydrops but rare.
followed by lethargy, poor feeding, altering of
conscious state, seizures and coma, is typical of Approach to the diagnosis of a metabolic
a) Organic acidemias including Maple syrup disease (Fig.1)
urine disease b) Urea cycle disorders. 1. History and clinical information
2) Energy deficient type with no window 2. Initial Screening or Primary laboratory tests
period. (Table II)
31
Acute neonatal encephalopathy
Non-metabolic causes Metabolic cause
Septicemia 1. Do Blood Ammonia

Meningitis
Hypoxic encephalopathy Very high Normal
Intracranial hemorrhage (>150um/L)
Hypoglycemia MSUD
NKH
2. Do ABG SOD
Peroxisomal disorders
Severe metabolic acidosis Normal/Resp. alkalosis
3.Do Lactate, Pyruvate, Ketones

Urea Cycle disorders
Mixed Keto-Lactic Acidosis Lactic acidosis only

Organic Acidemias
Propionic Acidemia
Methylmalonic Acidemia 4.Do Lactate Pyruvate Ratio
Isovaleric Acidemia
Multiple Carboxylase
deficiency Normal or low (<10) High(>30)
Pyruvate dehydrogenase deficiency 5.Beta(OH)Butyrate/AcetoAcetate ratio
< 2:1 > 2:1
Pyruvate Carboxylase def Resp Chain Defects

Fig 1. Approach to diagnosis Algorithm
i) CBC for neutropenia and thrombocytopenia lactic acidosis

ii) Electrolytes and arterial blood gas to iii) Blood sugar. Hypoglycemia indicates either
evaluate for metabolic acidosis and anion glycogen depletion +/- inadequate
gap gluconeogenesis (premature or SGA infant)
or hyperinsulinism (infant of a diabetic
Anion gap = (Na+ + K+) (Cl- + HCO3-) mother); occasionally hypoglycemia will be
Normal value = < 15 mEq/L a manifestation of a metabolic disease eg;
Metabolic acidosis with increased anion gap, fatty acid oxidation defect, glycogen storage
is suggestive of organic acidemias and primary disease
32
2003; 5(2):121
iv) Urine for ketones, reducing substance and vi) Uric acid is elevated in glycogen storage
ferric chloride test. Presence of urinary type Ia and low in molybdenum co factor
ketones is suggestive of organic acidemia defects.
and absence of which may denote fatty acid vii) Serum ammonia : If hyperammonemia
oxidation defects. If reducing substance in (>150umol/L) is detected in a newborn less
urine is positive, interpret it carefully, than 24 hours of age, think of transient
because of the chance of high false positivity. hyperammonemia of newborn, if preterm
Remember that glucose is a reducing and Pyruvate dehydrogenase deficiency
substance. So if the clinitest is positive (PDH),if full-term. After 24 hours of age,
(Clinitest detects all reducing substances hyperammonemia associated with keto-
including glucose), check the urine acidosis, is suggestive of Organic acidemias
specifically for glucose using a glucose and with normal blood gas or alkalosis is
oxidase strip. Urine dipsticks detect glucose suggestive of Urea cycle disorder.
only. Ferric chloride test for ketoacids is For accurate values, rapidly flowing blood
positive in Phenyl ketonuria, Tyrosinemia, (arterial stab) should be collected, placed in
Maple syrup urine disease, Histidenemia, ice and carried to the lab immediately. It
and Alkaptonuria. The test is non-specific should be done within 1 hour. Hence prior
and not used in modern laboratories. notification to the lab is necessary.
viii) Blood lactic acid, pyruvic acid and lactate
v) Low blood urea: Signifies urea cycle
pyruvate ratio.
disorders
If arterial lactate is persistently high
(>3mmol/L), the differential diagnosis is:
Table 2. Laboratory tests
Screening Laboratory Tests Confirmatory Tests

Disorders Blood gas Urine Lactic Serum Blood TMS Urine GCMS
Anion gap Ketones Acid Ammonia AA & Acyl (Organic Acids)
carnitine
MSUD - + - - + Not indicated
Organic Severe ++ + + + +
Acidemias acidosis
Primary Severe +/- +++ + + +
Lactic acidosis
Acidosis
Urea Cycle Resp - - +++ + Not indicated
Disorders alkalosis
Non-ketotic - - - - + Not indicated
hyperglycin
-emia
(+) abnormal test (-) normal test
AA-Amino acids; MSUD- Maple Syrup Urine Disease
TMS- Tandem mass spectrometry
GCMS - Gas chromatography & mass spectrometry
33
Table 3. Differential diagnosis of common metabolic disorders:

Diseases Clinical features Biochemical Confirmatory tests
abnormalities
Maple Syrup Urine smell, Neuro- Urinary Di-nitro phenylhydrazine;
Urine disease intoxication, tone ketones++ Aminoacid profile; TMS
changes, seizures Hypoglycemia+/-
(in first wk of life)
Organic acidemia Neuro-intoxication U.Ketones++ TMS
(within first month) Acidosis+++ Urine GCMS
Ammonia+
Lactate+
Pancytopenia
Urea cycle Intoxication type No ketosis TMS
disorders (in first week of life) Respiratory Aminoacid profile;
alkalosis Urine Orotic acid
Ammonia+++
Cong Lactic Energy deficiency Acidosis++ Lactate Pyruvate ratio;
Acidosis (since birth) Ketosis++ except B-OH.B/AA ratio;
in PDH Urine GCMS
NKH Energy deficient type, Normal CSF & Blood Amino acid profile,
SOD Myoclonic seizures, TMS, Urine Sulfitest; Plasma
Peroxysomal Severe hypotonia very long chain fatty acids
Disorders (since birth)
Galactosemia Hepatomegaly Hypoglycemia Enzyme studies
Fructosemia Hypoglycemia Cholestatic Urine GCMS
Tyrosinemia Jaundice, Fanconi jaundice;
syndrome (in first few Urine reducing
months of life) substance
Glycogen Hepatomegaly Hypoglycemia Enzyme studies
storage Hypoglycemia Lactate++
disease No jaundice Uric Acid+
Fatty acid Energy deficiency Non ketotic TMS
oxidation Hepatomegaly acidosis
defects (in first year) Hypoglycemia
TMS- Tandem Mass Spectrometry, GCMS-Gas chromatography mass spectrometry;
B-OH-B/AA Beta hydroxy butyrate and aceto acetate ratio;
NKH- Non-ketotic hyperglycinemia; PDH- Pyruvate dehydrogenase deficiency;
SOD- sulphite oxidase deficiency
34
2003; 5(2):123
a) severe organ dysfunction leading to decreased 3. CSF analysis for organic and amino acids
tissue perfusion/oxygen delivery or increased
metabolic demand as in perinatal asphyxia, Tandem mass spectrometry is the most
congenital heart disease (duct dependent lesions), significant advance in newborn screening in the
sepsis or untreated seizures. past 30 years 9 . While gas and liquid
chromatography is time consuming, using tandem
b) Primary lactic acidoses like disorders of mass spectrometry, more than 30 disorders can
pyruvate metabolism, mitochondrial disorders be easily detected from a drop of blood on filter
paper in a single test within 1 to 2 minutes. It
c)Secondary lactic acidoses Other metabolic detects molecules by measuring their weight
diseases may be associated with lactic acidosis (mass) electronically and display results in the
like fatty acid oxidation defects, organic acidoses form of a mass spectrum (graph showing each
etc. specific molecule by weight and how much of
ix) Urine for metabolic screen : 5-10 ml freshly- each molecule is present)10.
collected urine in a sterile container with no Definitive diagnostic tests include specific
preservatives; can be frozen prior to analysis enzyme analysis, assays for galactose 1-
if necessary. phosphate uridyl transferase , DNA testing on
Filter paper technique for collection of liver, muscle, or skin biopsy specimens.
capillary blood by heel prick: Spot 1 to 2 drops
Emergency management of a suspected
of blood onto each of the 3 circles on the specific
metabolic disease
filter paper provided, until the circles are filled
completely. Then let the blood spots to dry in It is essential that the treatment of patients
air. Blood spots can now be used for the diagnosis with inborn metabolic disease is started without
of a large number of inherited metabolic delay in order to avoid irreversible damage to
disorders. vital organs, especially the brain, and fatal
outcome in neonates where the clinical course
Before doing the primary investigations,
can be rapid.
ensure that the patient is well fed with an adequate
protein diet 2-3 hours before. Neonates should 1. Stop all oral feeds after collecting the necessary
be given milk feed. Primary investigations for tests. Collect 3 drops of blood on filter paper for
inborn errors of metabolism must be carried out Tandem MS. Collect 10 ml urine and deep freeze
as a matter of urgency and if indicated out of immediately for organic acids by gas
hour also. The specimens should be collected chromatography mass spectrometry (GCMS).
during the acute episode before starting treatment.
2. Monitor vital signs and biochemical parameters
If the above simple primary investigations periodically
are normal in a patient that is well fed, the chance
3. Treat sepsis if indicated.
of a metabolic disease as the cause of illness is
low. 4. Prevent dehydration and catabolism by giving
full maintenance fluid as 10% glucose with
Advanced Screening tests (Table 2) electrolytes
1. Tandem Mass Spectrometry (TMS)
5. Start next day intralipids and a small amount
2. Plasma Amino acid and urine Chromatography of amino acid mixtures (maximum 0.25 to 0.5
35
gm/kg/day) to prevent endogenous protein References

breakdown.
1. Chakrapani A, Cleary MA, Wraith JE. Detec-
6. Treat metabolic acidosis if pH <7.2 tion of inborn errors of metabolism in the new-
born. Arch Dis Child 2001; 84: F205-210
7. Hyperammonemia (Ammonia >150umol/L)
always warrant urgent treatment, as every minute 2. Applegarth DA, Toone Jr, Lowrt RB. Incidence
of Inborn errors of metabolism I British Co-
delay can cause neuronal damage.
lumbia, 1969-1996. Pediatrics,2000;105:10
Priming (stat) dose : 3. Burton BK. Inborn errors of metabolism in in-
Sodium benzoate 250mg/kg; Phenyl acetate fancy: a guide to diagnosis, Pediat-
rics,1998;102:69
250mg/kg; Arginine 660mg/kg (in urea cycle
disorders only) all diluted in 25ml/kg of 4. Ward JC. Inborn errors of metabolism of acute
10%Dextrose and infused over 90 minutes. onset in infancy. Pediatr. Rev,1990; 2:205
Maintenance dose: Same dose and dilution but 5. Walter JH. Inborn errors of metabolism and
infused over 24 hours. pregnancy. J Inherit Metab Dis, 2000; 23:229-
236
8. IV Carnitine. Useful in organic acidemias by
removing toxic organic acids and restoring 6. Diagnosis and treatment of Maple syrup urine
disease: a study of 36 patients. Pediatrics, 2002;
mitochondrial functions.
109(6):999-1008
9. Use of soluble insulin is anabolic and prevents 7. Irons, M. Screening for metabolic disorders.
hyperglycemia from use of high glucose How are we doing? Pediatr Clinics of North
concentrations (0.1 units/kg/hr with monitoring America, 1993; 40:1073-1085
of blood sugar)
8. Levy, H.Screening of the newborn. In Diseases
10. Specific treatment include cofactor biotin and of the Newborn. WB Saunders Co. Philadel-
Propimex-1 formula for Propionic acidemia, phia, 1991.6th ed: 111-146
Thiamine and Ketonex-1 formula for Maple
9. Naylor EW, Chace DH. Automated tandem
syrup urine disease, High carbohydrate diet and mass spectrometry for mass newborn screen-
biotin for Pyruvate carboxylase deficiency etc. ing for disorders in fatty acid, organic acid, and
amino acid metabolism. J Child Neurol,
Conclusion : IEM is an extremely challenging
1999;14 Suppl 1: S4-S8
area. For successful management, awareness,
early suspicion, and a good regional center with 10. Wiley V, Carpenter K, Wilcken B. Newborn
facilities for accurate early diagnosis and prompt screening with tandem mass spectrometry : 12
treatment including availability of special milk months experience in NSW Australia. Acta
formulas, are mandatory. Paediatr Suppl,1999; 88:48-51
ERRATUM
In the issue 2003;5(1), in page 81, under questions and answers column in the Answer 4
regarding need for Tet Vac, it should be read as Skunks instead of snakes and Ferrets
instead of parrots.
36
2003; 5(2):125
LABORATORY MEDICINE
LABORATORY DIAGNOSIS OF Lab diagnosis of persistent diarrhea

PERSISTENT AND CHRONIC
Patients with persistent diarrhea can be
DIARRHEA managed without elaborate laboratory tests using
* Shinjini Bhatnagar the above algorithm with very high levels of
success. Stool microscopy helps in identifying
Persistent Diarrhea trophozoites of E.histolytica and G.lamblia,
which are present in a very small proportion of
The duration of acute diarrhea forms a
children. Majority of children who have cysts of
continuum, most episodes terminating within 7
E.histolytica are now known to have non-
days and progressively smaller proportions
pathogenic E.dispar. Large number of pus cells
persisting beyond 14, 21 or 28 days. The
(> 20 /HPF) in stool suggest invasive diarrhea
delineation of persistent diarrhea from acute
but the specificity of this test is low and a majority
diarrhea is arbitrary. The most commonly used
of children with persistent diarrhea do not have
definition is an episode that begins acutely, is of
these.
a presumed infectious etiology and persists for
14 days or more. The pathogenesis is Stool cultures are expensive and not
multifactorial with persistent mucosal injury warranted in all cases. They should be ordered
being the hallmark of the disease. The common only when there is a high index of suspicion for
etiological factors are persistent or recurrent Shigella or Salmonella especially in very young
infection with enteropathogens namely Shigella, infants. Isolation of E.coli is not helpful as most
Salmonella, enteroadherant E.coli or small bowel laboratories cannot characterize for virulence
bacterial overgrowth. Secondary lactose or properties.
carbohydrate intolerance due to a combination
of macro and micronutrient deficiency and enteric In a non-hospital setting, detecting reducing
infection, and infrequently secondary milk substances is often impractical as tests cannot be
protein intolerance further perpetuates the done promptly and several stools need to be
mucosal injury and prolongs diarrhea. Several examined for sufficient sensitivity. In these
randomized controlled studies have shown that settings it is, therefore, more practical to use
antimicrobials offer modest or no clinical benefit clinical criteria to decide on change in diet as
in persistent diarrhea. Nutrition is the mainstay shown in the algorithm (Fig.1). The fact that
of treatment. A dietary algorithm using a stepwise diet-A has reduced lactose already assumes that
elimination of carbohydrates is recommended by some secondary lactose intolerance exists in
the WHO and the National Task Force on children with persistent diarrhea2. However,
diarrhea1. wherever possible stools should be tested for pH
* Centre for Diarrheal Diseases and Nutrition and reducing substances (see details of methods
Research, Department of Pediatrics, below) as it documents carbohydrate intolerance.
All India Institute of Medical Sciences, New
Delhi
37
Treat dehydration and any associated systemic infection

Start Diet A* [low lactose milk cereal mix]

Success Treatment failure

Start Diet B* [milk free; mixes of complex carbohydrates
disaccharides, milk free proteins and oils]

Discharge Success Failure

Start diet C* (monosaccharide based diets)

Resume appropriate diet for Start Diet C [After screening age 7-14 days later
& treating systemic infection]
*Screen and treat for systemic infection
Fig 1. Dietary algorithm for treatment of persistent diarrhea
A skilled microscopist and careful attention

Chronic Diarrhea to the collection and preservation of stool samples
is important for diagnosing enteric parasites.
Diarrhea that has persisted beyond 6-8 Presence of radio contrast material like barium,
weeks is defined as chronic and the etiology may antacids, mineral oil or antibiotics may interfere
differ by age3.b (Table 1) with the detection of protozoa and a period of 2
weeks without any of these substances would be
IMPORTANT LABORATORY
advisable before collecting the stools. Further,
DIAGNOSTIC STUDIES
contamination with water or urine can result in
Initial screening tests - Stool examination lysis of trophozoites. Examining multiple samples
obtained on separate days increases the sensitivity
The initial studies would include stool of detection because of variable shedding of cysts
examination for blood, leucocytes, protozoa and and trophozoites 4. Sensitivity of detecting
reducing substances. Identification of fecal Giardia trophozoites, cysts or antigens increases
leukocytes, red blood cells or occult blood by about 20% (about 70% if a single stool sample
suggests an inflammatory condition of the lower is examined) if three stool samples are examined.
colon. Fecal leukocytes are detected on Giardia is detected on direct smear or after
examining direct smears or after staining with concentration with 10% formol-ether.
3% Loefflers methylene blue. The smear should Examination of a fresh stool is ideal for
be allowed to stand for two or three minutes for identification of motile trophozoites.
good nuclear staining. All differential counts Routine ova plus parasite examinations do
should be made under high power, counting 200 not include tests for Cryptosporidium parvum and
cells when possible. Only those cells clearly other new enteric spore forming protozoa like
identified as either mononuclear or polymorho isospora and cyclospora and need to be specially
nuclear are included in the differential count. asked for. Cryptosporidium parvum occurs in
38
2003; 5(2):127
Table 1. Causes of chronic diarrhea in different age groups

Neonatal diarrhea C. difficile, (antibiotic-associated diarrhea)
Anatomic causes VIP secreting tumors
Immunodeficiency: common variable, severe
Congenital short-bowel syndrome
combined, X-linked agammaglobulinemia,
Malrotation with partial blockage
transient hypoglobulinemia
Hirschsprungs disease
Autoimmune enteropathy
Congenital microvillus atrophy
Tufting enteropathy Age 2 y to 18 y
Neonatal lymphangiectasia Celiac disease
Inherited transport defect Inflammatory bowel disease
Post-infective persistent diarrhea with Shigella,
Glucose-galactose malabsorption,
Salmonella, enteroadherant E.coli, Giardia
Congenital chloridorrhea,
lamblia, Cryptosporidium, small bowel bacterial
Bile-salt malabsorption
overgrowth
Milk enterocolitis
Chronic non-specific diarrhea (Irritable bowel
Age 1 mo to 2 y
syndrome)
Post viral or bacterial gastroenteritis e.g. lactose
Primary acquired lactase deficiency
intolerance, zinc and other nutrient deficiency
Tropical sprue
Persistent infection with Shigella, Salmonella,
Chronic pancreatitis/exocrine pancreatic
enteroadherant E.coli, Giardia lamblia,
deficiency
Cryptosporidium, small bowel bacterial
Primary or secondary lymphangiectasia, hypo/
overgrowth
abetalipoproteinemia
Cows milk, soy and other allergy
Acquired immunodeficiency
Celiac disease
Constipation with encopresis
Irritable colon of infancy (chronic non-specific
Antibiotic-associated C. difficile
diarrhea)
VIP secreting tumors7
Cystic fibrosis
upto 12% of immunocompetent children with Kinyoun acid fast stain

diarrhea and upto 24% in immunocompromised
hosts especially those with AIDS in developing Freshly passed stool is emulsified in 5 ml
countries5. At least 5-6 stools should be collected of 10% normal saline and filtered through two
in 10% formalin or sodium acetate-acetic acid- layers of gauze. 4 ml of solvent ether is added
formalin (SAF) on separate days. Staining with to the filtrate, mixed well and centrifuged at
modified Kinyouns acid-fast stain is usually the 1500 gyrations for 5 minutes. The supernatant
method of choice for clinical microbiological is decanted leaving 1-2 drops with the sediment.
laboratory while negative staining with Giemsa Smears are made from the thoroughly mixed
and concentration methods are restricted for sediment on glass slides, fixed in methanol after
research purposes6. A stool is considered positive air drying and are examined under oil
for Cryptosporidium parvum if typical oocysts immersion.
4-6 m in diameter are identified while the
cyclospora and isospora oocysts are larger and Enzyme linked immunoassay tests for
vary from 10-30 m in diameter respectively. antigens in stool for Giardia and Cryptosporidium
39
are highly sensitive and specific but are not Detection of reducing sugars in stool
routinely available.
1 ml distilled water is added to 0.5 ml liquid
Clostridium difficile diarrhea is uncommon stool and shaken well. Eight drops of this
in children but wherever the index of suspicion solution is added to 5ml of pre-boiled benedicts
is high, stool should be examined for the solution and is boiled for 1 minute. The solution
pathogen. The tissue culture assay for cytotoxin is cooled and the colour of the precipitate is
B is the gold standard but is expensive and examined:
cumbersome. Rapid toxin ELISA assay has The reducing sugars in stool are graded as
comparable sensitivity and specificity to those follows:
of the tissue culture assay and can be read within No colour change: nil
hours.
Green precipitate: 0.5%
Acidic stools with pH of less than 5.5 and/ Yellow precipitate: 1.0%
or positive for reducing substances usually Orange precipitate: 1.5%
indicate carbohydrate malabsorption and
Brick red precipitate: > 2.0%
proximal small bowel damage. Small bowel
mucosal injury results in malabsorption of For detection of non reducing substances in
carbohydrates. The unabsorbed carbohydrates in stool 1 ml N/10 HCl (instead of distilled water)
the small intestine are fermented by colonic is added to 0.5 ml liquid stool and boiled for 1
bacteria producing organic acids, carbon dioxide minute. The HCl splits the non-reducing to
and hydrogen gas. The organic acids get oxidized reducing sugars. The subsequent steps are as
and absorbed in the colon. This fermentative shown above.
action, which reduces the osmotic load of
particularly those who are exclusively breast-fed
malabsorbed carbohydrates and may benefit the
and pass liquid stools may be normal because of
host by salvaging calories, is known as the
a physiological malabsorption of lactose during
colonic salvage mechanism. Therefore the liquid
the neonatal period which has no nutritional
stool seen in carbohydrate intolerance is
significance.
characterized by an acid pH due to organic acids,
mainly lactic acid and by the presence of Lactose hydrogen breath test that detects the
unabsorbed sugars. The tests for identifying presence of hydrogen in the breath after giving a
acidic stools are valid only if the childs diet lactose dose of 2g/kg (to a maximum of 50g) is
contains sufficient quantities of carbohydrates. another useful diagnostic tool for documenting
Stool pH provides an indication to the amount of carbohydrate malabsorption. A rise in hydrogen
organic acids in stool while the increased amounts excretion greater than 20ppm 1-3 hours after the
of reducing substances indicate the presence of oral dose represents a positive peak and is
unabsorbed sugars. Sucrose is a non-reducing produced by the fermentation of unabsorbed
sugar and will react in this test only after it has carbohydrates by the colonic bacteria.
been acted upon by the colonic bacteria.
Detection of stool pH and reducing substances is Stool osmotic gap can be measured to
an important diagnostic tool in infants but may determine the osmotic nature caused by
not be helpful in older children as they have a unabsorbed carbohydrates. The osmolality
better colonic salvage mechanism. Presence of (mOsm/kg) and the fecal concentrations of
reducing substances in stool of neonates sodium and potassium (mEq/l) of a fresh liquid
40
2003; 5(2):129
stool sample is measured and the osmotic gap is Iron deficiency anemia is common in
calculated as: conditions where there is iron malabsorption or
chronic intestinal blood loss particularly in celiac
Osmotic gap = stool osmolality (approximately disease, inflammatory bowel disease and cystic
290 mOsm/kg) 2 (stool sodium + stool fibrosis. Megaloblastic anemia maybe present
potassium). There is an osmotic gap due to the in untreated celiac disease due to chronic
malabsorbed carbohydrates when the difference malabsorption of folate and B12. Inflammatory
is greater than 50 mOsm/kg. Stool sodium bowel disease may have anemia of chronic
concentrations greater than 90 mEq/L and an disease. Low levels of total protein and albumin
osmotic gap less than 50 mOsm/kg indicates a may reflect the nutrition status.
secretory diarrhea. These are specialized tests and Hypoalbuminemia may be seen in intestinal
maybe used in hospital settings in more lymphangiectasia. and inflammatory bowel
complicated cases to differentiate conditions disease. An elevated erythrocyte sedimentation
leading to osmotic (e.g. secondary carbohydrate rate suggests an inflammatory bowel disease.
intolerances, infrequently intestinal transport
defects) or secretory diarrhea (infections, Sudan III staining for fat malabsorption
infrequently congenital chloride and sodium
diarrhea or neural crest tumors). 2 drops of normal saline is added to one
drop of stool on a clean glass slide and is mixed
Sudan III stains fat globules in stool and is well with a stirring stick. Two drops of 95%
a quick and simple way to screen for fat ethyl alcohol is added to this mixture followed
malabsorption (Drummeys method). It is a by two drops of Sudan III stain. :Neutral fats
qualitative test and indicates gross steatorrhea. appear as orange or red drops.
Chemical analysis of fat in stool per 24 hours
collected over 72-hours using Van de Kamer A rough grading is as follows:
method gives an accurate quantitative Severe : > 100 big (> 6 m in diameter)
measurement of fat malabsorption. The child droplets / hpf
should be on a high fat diet (at least 50g/day) Moderate: < 100 big droplets /hpf
during the test. Co-efficient of fat absorption/ Mild : < 100 small (< 6 m in diameter)
retention can be calculated if the 72 hour dietary droplets /hpf
intake of fat is available as:
Fatty acids and soaps do not stain but appear as
(Dietary fat fecal fat) amorphous flakes or coarse crystals
X 100
Dietary fat Blood and urine d-xylose
The reference limits for fecal fat losses are D-xylose is a pentose sugar, which is
< 7% of daily fat intake for children > 6 months absorbed passively across the proximal intestinal
of age and < 15% of intake for younger infants. mucosa without requiring intestinal brush border
or pancreatic enzymes and bile salts. Its
Presence of severe steatorrhea would absorption is a measure of the functional surface
suggest an exocrine pancreatic deficiency. Mild area of the intestinal mucosa. A standard dose
or moderate fat malabsorption may be present in of 14.5g/m2 body surface up to a maximum of
chronic infections like giardiasis or in celiac 25 g is given by mouth. About 50% is
disease and other enteropathies. metabolized in the liver and the remaining is
41
Fig 2. Cryptosporidium oocysts seen in Fig 3. Characteristic fluorescent honey-

modified acid-fast stain combing seen around the smooth muscle
bundles on the monkey esophagus indicating
a positive antiendomyseal test on IFA assay
excreted in the urine. The serum d-xylose levels Characteristic histological features of celiac
of 25g/L at one hour and excretion of > 20% of disease include partial or total villus atrophy,
the oral d-xylose dose in urine collected for 5 elongation of the crypts, increased crypt mitotic
hours after giving the oral dose indicates a normal index, increased intra-epithelial lymphocytes
functioning mucosa (Reiners method). Although with a lymphocytic mitotic index above 0.2%,
d-xylose is a non invasive screening method for infiltration of plasma cells, lymphocytes, mast
assessing proximal intestinal mucosal surface cells and eosinophils in the lamina propria, loss
area, its low sensitivity and specificity limits its of nuclear polarity with pseudostratification of
use. It would be a good test for differentiating epithelial cells and absence of a brush border7.
between exocrine pancreatic dysfunction and an Well-defined histological features are seen in
abnormal intestinal mucosa. A normal d-xylose intestinal lymphangiectasia, giardiasis,
test in the presence of decreased serum pancreatic cryptosporidiosis (Fig 2.), abetalipoprotenemia,
enzymes like trypsinogen would suggest a acrodermatitis enteropathica. Cryptosporidium
pancreatic dysfunction. oocysts are identified on hematoxylin and eosin
stained sections as rows or clusters of spherical
SECOND PHASE TESTS structures attached to the microvillus border of
Intestinal biopsy the epithelial cells. In the small intestine the
lateral aspects of the villi have the maximum
Endoscopic biopsies are obtained from the number of oocysts. Significant mucosal
first part or midduodenum as compared to the inflammation indicates infections, immunode
earlier capsule biopsies, which were taken from ficiency, autoimmune enteropathy or protein
the dudeno-jejunal flexure. Intestinal biopsy is sensitive enteropathies. In the absence of an
an essential diagnostic tool in chronic diarrhea abnormal mucosal histology or inflammation,
and evaluates the crypt villus structure, epithelial exocrine pancreatic deficiency, hormonally
abnormalities and mucosal inflammation. mediated secretory tumors, colonic causes or
42
2003; 5(2):131
primary transport defects should be considered. colitis and helps in differentiating it from other
Colonic biopsies are essential for diagnosing colitides and also Crohns disease.
inflammatory bowel disease.
Testing for exocrine pancreatic insufficiency
Serology
Serological tests are important adjuncts in Quantitative measurement of 72-hour fecal
the diagnosis of celiac disease and are available fat collection that demonstrates less than 90%
in reference laboratories in our settings. Serum absorption of the fat ingested is a simple test to
IgG and IgA anti gliadin antibodies (AGA) were document exocrine pancreatic insufficiency.
the first generation serological antibodies and are These patients would have a normal d-xylose and
detected in an ELISA using crude gliadin extract. intestinal mucosa on biopsy9. Measurement of
The specificity of both IgG and IgA AGA is about fecal concentrations of the pancreatic enzymes
80% in studies done at our centre but the trypsin or chymotrypsin is an indirect measure
sensitivity of IgA is higher (90%) than that of of pancreatic function but these measurements
IgG (76%). IgA anti reticulin detected by are limited by proteolytic degradation. The
immunoflorescence (IFA) on rat kidney are ELISA for human fecal elastase is an alternative
highly sensitive (>90%) and specific (>90%) but fecal test of pancreatic dysfunction. It is
cumbersome and expensive. Serum anti IgA anti decreased in exocrine pancreatic deficiency. It
endomyseal antibodies (EMA) directed against has been seen that values > 100 g /g stool have
the reticulin like tissue around the smooth muscle 99% predictive value for ruling out pancreatic
fibres are detected in an IFA assay using the insufficiency 10 . Serum concentrations of
monkey esophagus (Fig 3). The sensitivity (91%) immunoreactive trypsinogen. determined by
and specificity (95%) of these antibodies is high radio immunoassay is a highly sensitive test for
in our settings and is consistent with the west. establishing pancreatic insufficiency but is not
Human umbilical cord is now being used as a very specific. Values < 28 ng/ml are suggestive
substrate instead of the more expensive and of pancreatic insufficiency. The gold standard
inaccessible monkey esophagus with similar remains the estimation of duodenal fluid enzymes
results. Because the test for EMA uses IFA it and bicarbonate collected after stimulation with
can be operator dependent and needs specialized secretin-pancreozymin or liquid feeding. Marked
laboratories and experienced hands. It may also reduction of the enzymes reflects severe damage
be less reliable in children less than 2 years. IgA to the acinar cells, usually when 60% of the
AGA and EMA are IgA dependent antibodies exocrine function is lost. Decreased levels of fat-
and will be negative in individuals with selective soluble vitamins and cobalamin indicate chronic
IgA deficiency, which is present in 3% of celiac pancreatic insufficiency.
disease. The most recent anti transglutaminase
(tTG) antibodies, which is now considered the Elevated concentrations of sodium and
main autoantigen for EMA, is measured by chloride in sweat stimulated by the pilocarpine
ELISA, a more objective and easily available test iontophoresis is an important diagnostic tool for
than the IFA8. Both guinea pig and human tTG cystic fibrosis. However in infants and during
have been used as antigens in the ELISA with the cold winter months collection of sweat maybe
results comparable to those of EMA in IFA assay. a problem. Further molecular diagnostic
Serological tests for detection of circulating techniques are used for confirming the diagnosis
pANCA are a useful diagnostic tool for ulcerative of cystic fibrosis.
43
THIRD PHASE TESTS 4. Hiatt Ra, Markell EK, Ng E. How many stool
examinations are necessary to detect pathogenic
Serum immunoglobulins and other intestinal protozoa? Am J Trop Med Hyg
extensive immunological tests to determine 1995;53:36-39.
immunodeficiency, measurement of anti- 5. Current WL, Garcia LS. Cryptosporidiosis.
intestinal epithelial antibodies in the serum in Clin Microbiol Rev 1991; 4:325-358.
suspected autoimmune enteropathy and
6. Chen X, Keithly JS, Paya C, LaRusso NF.
circulating VIP levels for neural crest tumors may
Cryptosporidiosis. N Engl J Med 2002; 346:
be required for a small number of patients.
1723-1730.
Special staining of biopsies and electron
microscopic evaluation is useful for diagnosis of 7. Bhatnagar S, Cameron DJS, De Rosa S, Maki
microvillus inclusion disease and tufting M, Russell GJ, Troncone R. Recommendations
of the Working Group on Celiac Disease. J
enteropathy.
Pediatr Gastroenterol Nutr 2002; 35(2): S78-
References 88.
8. Molberg O, Mcadam S, Korner R, et al . Tis-
1. International working group on persistent di-
sue Transglutaminase selectively modifies glia-
arrhoea. Evaluation of the efficacy of an algo-
din peptide that are recognized by gut derived
rithm for the treatment of persistent diarrhoea :
T-cells in celiac disease. Nat Med 1998; 4:713-
A multicentric study. Bull WHO 1996;74:479-
717.
489.
9. Toskes PP, Greenberger NJ. Disorders of the
2. Bhatnagar S, Singh KD, Sazawal S, Saxena SK,
pancreas. In: Harrisons principles of internal
Bhan MK. Efficacy of milk versus yogurt feed-
medicine, 15th edn, eds Braunwald E, Fauci A,
ing in acute non-cholera diarrhoea among mal-
Kasper D, Hauser S, Longo D, Jameson J. ,
nourished children. J Pediatr 1998; 132:999-
McGraw Hill Companies, USA (pub), 2001;
1003.
pp 788-1792.
3. Vanderhoof JA. Diarrhea. In: Pediatric gas-
10. Bebarry S, Ellis L, Corey M, Marcon M, Durie
trointestinal disease:pathophysiology, diagno-
P. How useful is fecal pancreatic elastase 1 as
sis, management, Wyllie R, Hyams JS (ed). WB
a marker of exocrine pancreatic disease. J
Saunders company, USA, (pub) 1999, p-32-42.
Pediatr 2002;141:84-90.
NEWS AND NOTES

PAED ENDO- 2003
Paediatric Endocrinology for Practicing Paediatricians
and Postgraduates - An Update
Organised by
Paediatric Endocrinology Division- Institute of Child Health and Hospital For Children, Chennai-8
Indian Academy of Pediatrics-Chennai City Branch
Indian Academy of Pediatrics-TNSC
Date : 26th July 2003 - Saturday Delegate Fee Rs. 300/-
Venue : Savera Hotels Ltd., Dr. Radhakrishnan Salai, Chennai - 600 004.
Delegate Fee (Cash, Cheque Or Draft) to be drawn in favour of Paed Endo and send to
Dr P. Venkataraman, Organising Secretary-Paed Endo 2003
IAP TNSC Flat, F Block, Ground Floor, Halls Towers, 33, Halls Road, Egmore, Chennai- 8
Phone: 28190032, 28191524 Cell:98401 19237
44
2003; 5(2):133
LABORATORY MEDICINE
LABORATORY EVALUATION OF secondary unless proved otherwise and every step

HYPERTENSION should be taken to find out the cause.
Management of the cause is mandatory in
addition to management of hypertension.
* Vijayakumar M Treating elevated BP without managing the cause
** Prahlad N if treatable will not make proper sense.
*** Nammalwar BR
Blood pressure is an important basic
Introduction physical sign as are the body temperature, pulse
rate and respiratory rate. The measurement of
The interest of pediatricians in
blood pressure is now firmly established as an
hypertension has increased markedly in the last
essential component of routine pediatric physical
two decades essentially due to recent
examination. It is mandatory for every child,
developments in the field of medicine. Primary
three years of age and older to have as a part of
hypertension, which was considered a disease of
routine continuing medical care, a yearly blood
an adult, is also being documented in children.
pressure measurement. In addition acutely ill
Essential hypertension in adults may be preceded
children, regardless of age should have a blood
by high BP of childhood. Control of blood
pressure reading performed at the time of
pressure in children and hence, prevention of end
evaluation. Investigation of a child with
organ damage in adulthood can be done easily.
hypertension depends not only on the severity of
Lifestyle modification in terms of diet, salt
hypertension but also whether the child is
restriction, exercise and stress can be introduced
symptomatic and hypertension is the suspected
early in childhood to have full benefit in
cause. Possible investigatory procedures range
adulthood. Hence, measuring BP regularly as a
from routine tests available in most hospitals to
part of routine care of each child and adolescent
more refined invasive procedures available in
should be the rule in pediatric care. Finding out
specialized units1.
the cause for hypertension is the most important
aspect of management. Detailed clinical history, Goals of evaluation
essential clinical examination, initial There are five major goals of initial
investigations followed by appropriate evaluation of hypertension in children2,3.
investigations based on clues obtained from the
previous steps are needed. Cause of hypertension 1. To establish whether hypertension is sustained
in children should be always considered and might benefit from treatment
2. To identify coexisting diseases
* Pediatric Nephrologist,
3. To characterize the risk factors
** Fellow in Nephrology,
4. To identify the presence and severity of target
*** Chief Nephrologist
organ damage
Kanchi Kamakoti CHILDS Trust Hospital,
5. To identify curable causes of the hypertension
Chennai 600 034.
45
Definition of hypertension4 Table 1. Common causes of childhood

hypertension
Normal BP: Systolic blood pressures (SBP) and
diastolic blood pressures (DBP) below 90 th Neonate
percentile for age, sex and height.
Renal malformation
High normal BP: Average SBP and/or average Coarctation of aorta
DBP between 90th and 95th percentile for age, sex
Renovascular hypertension following
and height.
umbilical artery catheterisation
High BP (Hypertension): Average SBP and/or Bronchopulmonary dysplasia
average DBP above 95th percentile for age, sex Infancy to 6 years of age
and height on three occasions.
Renal parenchymal disease
Causes of hypertension in children Renal artery stenosis
Coarctation of aorta
One should know the common causes of
hypertension in various age groups to decide on 6-10 years of age
diagnostic evaluation (Table 1)4. Laboratory Renal parenchymal disease
evaluation should be preceded by detailed history Renal artery stenosis
and clinical evaluation to get clues on the cause
Endocrine causes
and hence to decide on investigations.
Primary or essential HT
95% of hypertension in children is acute Adolescent hypertension
hypertension, wherein the cause is obvious. In
sustained hypertension, i.e., hypertension Renal parenchymal disease
persisting for more than 6 weeks and/or with Essential hypertension
echocardiographic evidence of left ventricular
hypertrophy, 95% are secondary to an underlying Measurement of blood pressure in all the 4 limbs
renal/extrarenal cause. It is this group which is vital and auscultation for bruit, however
needs evaluation for diagnosis. In the majority obvious the diagnosis may be, should not be
of them, the diagnosis will be obvious with a good ignored.
clinical history and thorough physical Investigations in hypertension1,4,5
examination. More often, investigations are
necessary to confirm the clinical diagnosis and In clinical practice, the following
in only about 5% of patients, investigations are investigations are necessary and more often
necessary for diagnosis itself. Hence, the clinician sufficient to make a diagnosis.
must lay emphasis on proper history elicitation
and complete physical examination which is i. Urine analysis
outside the purview of this review. History should ii. Urine culture (with proper precautions)
include family history of hypertension,
iii. Serum creatinine
cerebrovascular accidents, renal death and sudden
death. Physical examination should include skin iv. Serum electrolytes
changes like caf-au-lait macules, v. Ultrasonogram of abdomen
neurofibromatosis, renal and bladder mass.
vi. Echocardiogram
46
2003; 5(2):135
vii. Renal biopsy (in the event of diagnosis of X-ray chest and abdomen
chronic glomerular disease)
Cardiomegaly and left ventricular
The rest of the investigations discussed hypertrophy are the important features of
below along with common investigations sustained hypertension. X-ray abdomen may
mentioned above need the assistance of pediatric show features of renal stones and pointers for
nephrologist. metabolic renal bone disease.
Urinalysis Echocardiography
Severe proteinuria and hematuria suggests On documentation of sustained hypertension

glomerulonephritis. Hypertension of long the child should undergo echocardiography to
standing nature can produce mild to moderate assess the end organ effect of hypertension, left
proteinuria without hematuria. Low specific ventricular hypertrophy. Further, coarctation of
gravity or osmolality may be seen in chronic aorta as the cause for sustained hypertension can
tubulointerstitial disease, chronic renal failure, be identified. Serial echocardiographic
renal cystic disease and dysplasia. Urinalysis evaluation periodically is mandatory to ascertain
may be normal in renovascular hypertension. the effect of therapy.
Transient urinary findings, may make one miss Ultrasonogram of abdomen
the diagnosis in PIGN.
This imaging modality is popular for
Blood count documenting the renal size, gross anatomy and
intrinsic details of kidney structure. Multiple
Microangiopathic hemolytic anemia is a communicating cystic structures, large renal
feature of hemolytic uremic syndrome and pelvis and visible parenchyma are features of
normocytic normochromic anemia is seen in hydronephrosis. Diffuse echoes or echogenic
chronic renal failure. mass within the vessels may denote renal artery
Routine serum chemistry or renal vein thrombosis. Reflux nephropathy
shows irregular small kidneys. Chronic
Serum creatinine and blood urea estimation pyelonephritis due to both reflux and non-reflux
help in identifying renal impairment of renal causes can show small and irregular kidneys.
parenchymal disease. Elevated blood glucose can Bilateral smooth and small kidneys indicate
be seen in pheochromocytoma. Serum chronic glomerulonephritis or renal artery
cholesterol and triglyceride estimation are stenosis, hypoplasia or dysplasia involving both
important, as they are cardiovascular risk factors. kidneys. Unilateral small regular kidney indicates
unilateral renal artery stenosis. Infantile
Normal serum electrolyte rules out adrenal polycystic kidneys are identified by echogenic
hormonal disturbance as a cause for hypertension. enlarged kidneys with small cysts.
Low serum sodium with elevated potassium Nephromegaly with multiple large cysts denote
suggests hypoaldosteronism or congenital adrenal autosomal dominant polycystic kidney disease.
hyperplasia. Hypokalemia, metabolic alkalosis
and high normal serum sodium indicate Intravenous urogram
hyperaldosteronism. Hyperkalemic metabolic This imaging modality is important for
acidosis is a feature of renal failure. assessing renal size anatomy and function of
47
individual kidneys. A conventional IVU is of little anatomy is delineated and is useful for PUV
value and only minute sequence IVU is found detection. We can also grade the VUR but the
useful in childhood hypertension. In conventional test cannot be repeated frequently, as ionizing
IVU we take the first picture after the contrast radiation is more. If Dimercapto Succinic Acid
only at 5th minute. On the contrary if we do Scan (DMSA) is indicative of pyelonephritis but
minute sequence (rapid sequence) IVU with voiding cystourethrogram (VCU) failing to show
pictures after contrast at 1, 2, 3 and 5 minutes VUR the child may need direct radionuclide
followed by regular IVU pictures one can cystogram (DRNC), which is more sensitive than
document the ischemia effectively. Distal branch VCU to detect VUR. Direct radionuclide
artery stenosis may not be picked up in IVU. On cystogram can be repeated frequently, as ionizing
documenting ischemia selective renal radiation is less.
angiography can be done using concerned
modalities. Unilateral renal artery stenosis can Radio nuclide imaging
be identified by diminished size of the kidney, Radio nuclide scintigraphy may be used to
delay in appearance of nephrogram and delayed image genitourinary tract and to examine renal
excretion of the contrast by the ischemic kidney, perfusion and functioning. Radionuclide
the kidney with renal artery stenosis. Apart from scintigraphy uses pharmaceutical compounds that
these three major criteria for ischemic kidney can assess renal function and anatomy by
there are many minor criterias. Decrease in the measurement of GFR, tubular secretion or tubular
size of the pelvicalyceal system, ptosis of the integrity by measurement of isotope retention.
kidney, notching on the ureters due to collaterals Radionuclide renal scans have greatly reduced
developed following renal ischemia are some of the need for intravenous urogram as an
them. investigation in childhood hypertension. In this
Apart from renal ischemia, reflux modality the child is exposed to only a small dose
nephropathy can be documented by abnormal of ionizing radiation. Information on renal blood
renal contour and deformed calyx indicative of flow and split renal function can be obtained.
renal scars which is also the feature of chronic Even in children with compromised renal
pyelonephritis due to non-reflux causes. Opaque function this test can be done. This test has
striations running into cortex indicative of completely replaced the need for IVP in neonates.
autosomal recessive polycystic kidney disease IVP in newborns is associated with poor
and distorted, splayed calyces due to multiple concentration of the radio-contrast by the
cysts denoting autosomal polycystic kidney kidneys. But one should note that urinary tract
disease can be noted in IVU. anatomy is not better described by radionuclide
scan.
Voiding cystourethrogram (VCUG)
In children with renovascular hypertension
This important imaging modality is needed the renal radionuclide scan (DTPA) shows
to document posterior urethral valves (PUV) and reduction in renal blood flow and GFR on the
vesicoureteric reflux (VUR) without ambiguity. affected side in the renogram. On doing captopril
Reflux nephropathy causing hypertension and renogram after one hour of challenge dose of
PUV related chronic tubulointerstitial disease are captopril, the reduction in blood flow and GFR
some of the common causes of hypertension in are found accentuated in children with
children. In conventional VCUG urethral renovascular hypertension. Bilateral
48
2003; 5(2):137
renovascular disease is difficult to diagnose with Selective renal angiography

captopril enhanced renal scan as the blood flow
is symmetrically diminished on both sides. This study is essential to localize the site of
renovascular disease and to predict the type of
In DTPA the delay in tracer reaching the disorder causing the renovascular hypertension.
collecting system as noted by delayed intrarenal Fibromuscular dysplasia which is a common
transit time suggest i) renal insufficiency ii) renal etiology of renovascular hypertension is
vein thrombosis iii) pyelonephritis iv) renal artery characterized by stenotic lesions in the main renal
stenosis and v)dysplastic kidney. When there is artery and post-stenotic aneurysmal dilatation.
normal transit time but delay in drainage the likely Beaded appearance in the renal artery is possible
possibility could be PUJ obstruction or VUR. In if several segments are involved. In aortoarteritis
ureteropelvic junction obstruction delay in the renal arteries are usually involved at the origin
excretion noted in the excretory phase not from aorta.
abolished by diuretics is indicative of organic
Digital substraction angiography can replace
obstruction.
selective angiography as the radio contrast is
injected intravenously in a large vein and the
Cortical function defects seen in
arterial phase of renal vasculature is obtained by
pyelonephritis, infarction, scarring and fetal
digital substraction methodology. This method
lobulation are identified by DMSA. Reflux
needs larger volumes of radio-contrast material
nephropathy is the common condition associated
and cooperation of the patient. Because of
with scarring. It also helps in identifying the
smaller sized renal arterial tree, this method is
cortical function both in acute and chronic
not fully useful in children.
pyenonephritis even in children with renal failure.
Classical aortogram done with contrast
Computerised tomography of kidneys followed by selective renal angiography was the
gold standard investigation for renovascular
This essential modality of imaging is
hypertension in adults. This investigation was
very useful in picking up tumours associated with
done with great difficulty in children above 5
hypertension and perirenal collections causing
years of age. The advent of doppler study, CT
ischemia to the kidney as in Page kidney. The
angiography and MRI angiography has
tumours like neuroblastoma and pheochromo-
revolutionized the investigation for evaluating a
cytoma can be identified.
child with renovascular hypertension.
Doppler flow ultrasound MRI arteriogram
This study is attractive by being non- It is useful in the evaluation of renal arterial
invasive but is not sufficiently sensitive to be the vasculature and aorta and to assess the function
final diagnostic imaging study when renovascular of renal hypoperfusion. It helps to identify renal
disease is suspected. Positivity is very useful but arterial narrowing due to intraluminal and
negativity does not rule out renal ischemia. extraluminal causes. Advantage of MRI
Increased peak velocity, spectral widening distal arteriogram is that contrast needed is much less
to the stenosis are the features documented in and hence less nephrotoxicity. The sensitivity
renal artery stenosis and in renal transplant artery and specificity is less when compared to CT
stenosis. angiography.
49
CT angiography hyporeninemia along with hyperkalemic

hypertension, hyperchloremia and normal renal
The sensitivity and specificity of this function may point towards Gordons syndrome.
important imaging modality is 98% and 94% With hypoaldosteronism with hypokalemic
respectively. It is very safe procedure in children hypertension one should think of
and is less invasive than digital substraction psuedohyperaldosteronism, Liddles syndrome.
angiography.
Serum cortisol and 24 hours urine 17 hydroxy
Renal vein renin assay corticosteroid
This assay is usually done along with renal Elevated free serum cortisol and 24 hours
angiographic study. Renal vein renin level is 25% urinary 17 hydroxy corticosteroids are suggestive
higher than peripheral venous renin level. In of hypercortisolism. Following dexamethasone
children with unilateral renal or renovascular (3.75 mg/m2/day x 2 days) if the level falls it is
disease, renal vein renin levels on the affected suggestive of ACTH induced adrenal hyperplasia
side is exceedingly high. Renal vein renin level (Cushing disease).
from the diseased side should be above 50% than
renin level of inferior venacava and then only Urinary and plasma catecholamine levels
full benefit will be present after intervention.
Elevated plasma epinephrine or
Peripheral plasma renin activity norepinephrine or increased urinary
metanephrine levels suggest pheochromocytoma
Elevated levels are seen in renovascular and neuroblastoma.
hypertension, renal parenchymal disease and
some of those with essential hypertension. A very Meta iodo benzyl guanidine (MIBG) scan
low peripheral plasma renin activity is seen in
mineralocorticoid excess hypertension. Elevated It is both sensitive (86%) and specific (95%)
plasma renin levels is associated with renal artery in identification of catecholamine secreting
stenosis and 21 hydroxylase deficiency. Primary tumours like pheochromocytoma. A reimaging
reninism is caused by renin secreting tumours, after 48 hours of injection of radionuclide
benign or malignant, which can be intrarenal or material can also identify extrarenal location.
extrarenal. All of them have hypokalemia with Approach to investigation in hypertension4,6
elevated plasma renin levels and can be
diagnosed with angiography and CT scan. Confirm BP on three occasions. If normal
BP is documented (BP < 90th percentile), we
Plasma aldosterone are going to maintain the health surveillance.
Elevated aldosterone level is associated with If hypertension is confirmed (BP > 95th
elevated plasma renin in renal artery stenosis. In percentile) we go to the next stage.
hyperaldosteronism, the elevated aldosterone is History of drug intake, decongestant nasal
noted with normal renin level. Low aldosterone sprays or oral decongestants
along with high plasma renin is noted in 21 (sympathomimetics) for the common cold
hydroxylase deficiency. Low aldosterone and and glucocorticoids will give the clue
low renin levels are seen in 11 beta hydroxy towards drug induced hypertension. Stop
steroid dehydrogenase and 11 hydroxylase the drug and reevaluate after adequate
deficiencies. Hypoaldosteronism with period. If hypertension is resolved, a drug
50
2003; 5(2):139
induced/related hypertension is the diagnosis essentially renal parenchymal hypertension.

made. If hypertension is not resolved, child History and clinical examination by this time
needs investigations for other secondary also would have pointed towards the etiology
etiologies. Similar step also should be taken of renal parenchymal disease either
when there is no history of drug intake in glomerulonephritis or the other group
the hypertensive child. including chronic pyelonephritis, renal
dysplasia and cystic renal disease.
Documentation of BP in all four limbs is an
important step. Upper extremity Investigations needed in glomerulonephritis
hypertension only, will give us the clue are:
towards coarctation of aorta. Documentation
24 hours urine protein, creatinine and
of pulses in peripheral, palpable and
creatinine clearance
accessible blood vessels is mandatory.
Hypertension in all the four limbs will Serological evidence for infection in acute
indicate the need for investigatory approach HT like ASO
for other secondary causes for hypertension.
Complements C3, C4 in immune related
ECHO evaluation, doppler studies and
hypertension
aortogram may be needed as per the
diagnosis. Serological tests for SLE
If plasma renin activity is low, one should Peripheral blood smear for microangiopathy
consider mineralocorticoid excess. If it is for acute HT
high one should think of renovascular
Renal biopsy in non-contracted kidneys for
etiology (or even renal disease causing
confirming the diagnosis of chronic
hypertension). Renal ultrasound, captopril
glomerular disease
scan, renal vein renin, doppler study and
renal angiography are the investigations Investigations for chronic pyelonephritis,
needed in this situation. IVU will give renal dysplasia and cystic renal disease
positive findings of ischemia in the kidney include:
that has affected blood vessel. But Renal ultrasound, Radionuclide renal scan,
assessment of plasma renin and renal vein DTPA and/or DMSA scans, VCUG in
renin are not routinely available in many chronic pyelonephritis
centers. Discrepancy of kidney size by USG
and ischemic changes in the affected kidney In children showing abnormal urinalysis
by minute sequence IVU will point towards (hematuria and/or proteinuria or defective
renal artery stenosis. urinary concentration) renal disease as the
cause of hypertension should be considered
Renal function tests like estimation of blood
and investigations for glomerulonephritis
urea, serum creatinine and serum electrolytes
and the group of chronic pyelonephrits, renal
are mandatory. If the child has normal renal
dysplasia and cystic renal disease should be
function but hypokalemia one should think
done depending on the presentation of other
of mineralocorticoid excess. Even
features.
renovascular hypertension can have
hypokalemic hypertension. If the child has A clinical suspicion of pheochromocytoma
abnormal renal function the diagnosis is should make the clinician do the relevant
51
investigations. Sustained or labile References

hypertension, poor weight gain, episodes of
1. Rita D Swinford, Julie R Ingelfinger. Evalua-
shock like features needing intravenous
tion of hypertension in childhood. In: Pediat-
fluids, excessive appetite, hypertensive crisis ric Nephrology, 4th Edn., Eds., Martin Barrat,
on palpation of abdomen are some of the Ellis D.Avner, William E. Harman, Lippincott
features that make one think of William & Willkins, Pennsylvania 1999; pp
pheochromocytoma. Relevant investigations 1007-1037.
include urinary catecholamines and 2. Kishore Phadke. Investigations in hyperten-
metabolites, plasma catecholamines, sion in children. In: Pediatric Nephrology, 2nd
computerized tomography of the abdomen Edn, Eds. Nammalwar BR, Vijayakumar M.
and 131I metaiodobenzyl guanidine (MIBG) Madras, 1991; pp 303-305.
scan to confirm the cause of HT. 3. Srivastava RN, Bagga A. Hypertension. In:
Pediatric Nephrology, 3rd Edn Eds. Srivastava
Conclusion
RN, Bagga A, JayPee Brothers, New Delhi,
Hypertension in children is no longer an 2001; pp 228-242.
uncommon disease. Causes are many. 4. Kanwal K.Kher. Hypertension. In: Clinical
Hypertension in children should be always Pediatric Nephrology, International Edition,
considered secondary unless proved otherwise. Eds Kanwal K.Kher Sudesh P.Makker,
Mcgraw-Hill Inc. New York 1992; pp 323-375.
Essential hypertension is also possible in children
and is being increasingly documented in 5. Pankaj Hari, Rajendra N, Srivastava. Renal
adolescents. High normal BP of childhood is a imaging in children with persistent hyperten-
sion. IAP J Pract Pediatr 1996; 4(4): 237-244
pointer towards adult hypertension. All possible
investigations should be done as per history, 6. Vijayakumar M. Investigatory approach to
childhood hypertension. Proceedings of the
clinical examination and initial laboratory
CME programme on Pediatric Nephrology-
evaluation before defining hypertension as 2002 and beyond of National Academy of
essential. Medical Sciences. 2002; pp 80-82.
NEWS AND NOTES

Lakeside Education Trust, Twenty First Annual CME
Subject: Recent trends in pediatric practice
Date: - Sunday, July 27th, 2003. Venue: Hotel Atria, Bangalore.
For further details contact
Dr. H. Paramesh, Chairman, Lakeside Education Trust,
21st Annual CME Secretariat, Lakeside Medical Center and Hospital
33/4. Meanee Avenue Road, Near Ulsoor Lake, Bangalore - 560 042
Phone: 5303677, 5304276, 5566738, 5366723, 5512934; Fax: 5303677
E-mail: dr_paramesh [email protected]
Co-sponsored by
H.P. Foundation, IAP Karnataka State, IAP - Bangalore Branch (BPS), IMA - Bangalore East Branch
Pediatric Association of Bangalore, Lakeside Medical Center & Hospital,
IAP Environment & Child Health Group, Karnataka Nephrology Transplant Institute
Lakeside Institute of Nursing & Medical Technology
52
2003; 5(2):141
LABORATORY MEDICINE
CLINICAL NEUROPHYSIOLOGY clinical neurophysiologist on the other hand

IN PEDIATRIC PRACTICE should have adequate training in the different
modes of testing and the interpretation of the test
* Ayyar SSK results and aid in advancing and clinching the
* Suresh Kumar diagnosis. In short, any neurophysiology test
** Vasanthi D should be considered as an extension of the
*** Sangeetha V clinical neurological examination.
The vast advances in medical electronics and EEG (Electroencephalography):- Chief role is
computer technology have made several in the diagnosis of epilepsy and definition of the
sophisticated but essential investigations possible seizure type. Epileptiform discharges are
in the diagnosis, management and prognosis of recorded in about 45-50% cases of epilepsy; with
neurological disorders. activation techniques such as hyperventilation,
photic stimulation, sleep deprived and sleep
A well-equipped neurophysiology induced recordings the yield of information can
laboratory caters to a multitude of test protocols, be increased (Fig 1 and 2). Nevertheless it should
the common among them being EEG be remembered that a normal EEG does not
(electroencephalography), EMG (electromyo exclude epilepsy and diagnosis of epilepsy should
graphy), NCS (nerve conduction study- motor always be clinical at the first instance. Advances
and sensory), VEP (visual evoked potentials), in technology have made it possible to integrate
BSEP (Brainstem auditory evoked potentials), EEG and video imaging of the patient so that the
BERA (Brainstem evoked response audiometry), epileptic discharge can be synchronized to the
SSEP (Somatosensory evoked potentials both epileptic fit as observed on the video
neural and dermatomal stimulation), RNS (synchronized video EEG). Such precise
(Repetitive nerve stimulation study for correlation between the seizure and epileptic
myasthenia), Facial nerve and blink reflex studies discharge has become mandatory and a prelude
and SSR (sympathetic /galvanic skin response). to the consideration of epilepsy surgery.
Such an array of tests demand precise EMG (Electromyography) uses a needle
knowledge of neuroanatomy and electrode inserted into the muscle which records
neurophysiology on the part of the technician and the electrical potentials generated in the muscle
hands-on experience in the performance of the at rest and during graded muscular contraction.
tests in order to reach a degree of perfection. The A comparison is then possible between normal
* Consultant in Clinical Neurophysiology pattern and values and those obtained in diseases
** Senior Neurotechnician of the muscle such as muscular dystrophy,
*** Junior Neurotechnician myopathy (congenital, acquired), motor neuron
Neurolaboratory and Epilepsy & Sleep
disease and peripheral nerve diseases. Fig 3 and
Disorders Centre, Vijaya Health Centre, 175, 4 compare the findings in motor neurone disease
NSK Salai, Vadapalani, Chennai - 600 026 and myopathy.
53
FP2-A2
F8-A2
T2-A2
T4-A2
T6-A2
O2-A2
Fp1-A1
F7-A1
T1-A1
T3-A1
T5-A1
O1-A1
Fig 1. 3Hz spike and slow wave discharge elicited on hyperventilation in petitmal seizures
F p 2-A 2
F 4-A 2
C 4-A 2
P 4-A 2
O 2-A 2
F p 1-A 1
F 3-A 1
C 3-A 1
P 3-A 1
O 1-A 1
Fig 2: Generalised spike and slow wave complexes frontally predominant in a patient with
generalised tonic clonic seizures
54
2003; 5(2):143
E M G - LeftA BD D igM inim i E M G - LeftA BD D igM inim i
p o sitive sh a rp w a ve
fib rilla tio n s
100 (V ) 10 (m s 500 (V ) 50 (m s
Fig 3: EMG in motor neurone disease classical findings are fibrillations, positive sharp waves, long
duration high amplitude motor unit potentials, delayed recruitment and reduced interference pattern
E M G - R ightD eltoid E M G - R ightE xtD ig Brev
200 (V ) 10 (m s 200 (V ) 50 (m s
short duration MUPS low amplitude interference pattern

Fig 4: EMG in myopathy: The characteristic finding is motor unit potential (MUP) of short duration
and low amplitude. As all motor neurons are intact the interference pattern is full. The recruitment of
motor units is early in these cases
NCS (Nerve conduction study): The peripheral conduct impulse to the muscles and end organs
nerves for the four limbs and trunk emanate from and the sensory fibres conduct impulse from the
the spinal cord and the cranial nerves from the peripheral sense organs to the central nervous
brainstem. The motor fibres in these nerves system. Nerve conduction study evaluates the
55
conduction of the nerve impulse in terms of and H reflex). NCS is the most common
latency (time from stimulus to the onset of neurophysiological evaluation in neurological
response), velocity of conduction and amplitude practice and is useful in a variety of peripheral
(voltage of the motor or sensory action potentials nerve disorders such as diabetic neuropathy,
generated). The conduction in proximal portions nutritional neuropathy, Guillain Barre Syndrome,
of the peripheral nerves and roots can be toxic and hereditary neuropathies. (fig 5, 6 & 7)
measured by the late response studies (ie, F wave
R ightM edian M otor R ightM edian M otor

P NORM Al HM SN
D L - 2 .5 8 A m p 1 4 . 3 4 m V D L - 6 .5 m s, A m p - 0 .0 6 m V
P L 6 .0 2 C V 5 2 .3 3 M /s P L -1 4 .7 7 , C V 1 3 .3 1 M /s
O W rist P
W rist
R
O TR
T
P
P
Elbow
O
TR
Elbow
O R
2000 (V ) 5 (m s) 50 (V) 5 (m s)
Fig 5: Hereditary Motor Sensory Neuropathy (HMSN) Type 1 in a girl aged 6 yrs; Note
prolonged distal latency, grossly reduced amplitude and grossly decreased motor conduction
velocity. Normal response and values are depicted on the (L) side
R ightPeroneal-F R ightPeroneal-F
NO RM AL
F la te n cy 4 1 -4 6 m s
2000 (V ) /200 (V ) 10 (m s 1000 (V ) /100 (V ) 10 (m s

Fig 6 : Guillain Barre Syndrome (GBS) boy of 16 yrs; the earliest abnormality is
prolongation, absence or infrequency of F wave response and this is the hall mark of GBS in the
typical clinical setting
56
2003; 5(2):145
LeftTibialH R ightTibialH
H - R e so n se N O R M A L
H - R e sp o n se A B S E N T
M
20000 (V ) /1000 (V ) 10 (m s 500 (V ) /500 (V ) 10 (m s

Fig 7 : H reflex response from a normal subject compared to absent H response in sciatic
nerve injury following gluteal injection in a boy of 6 yrs
EP (Evoked Potential Studies): Advances in the retina of the eye to reach the visual area of
electronic and computer averaging techniques the brain concerned with normal perception
have made it possible to measure very tiny signals (P100 latency). This test is of immense value in
of the order of microvolts (one millionth of a volt) assessing the visual pathways in conditions such
from the visual, auditory and somatosensory as optic neuropathy (Fig 8), multiple sclerosis,
pathways in the central nervous system. pituitary and other brain tumours.
Measurement of the artifact-free biological signal
obtained is made in terms of latency (time period VEP
from stimulus to response) and amplitude of the N 75
N 145
signal. Sequential recordings from different 1

1:
2R
levels of the spinal cord (spinal evoked potentials)
makes it possible to assess conduction in N o rm al
segments of the spinal cord and helps in the P 100 R 112 m s 13.6 u v
localization of the spinal lesion. 1:

4L
N 145
N 75
1:
3L
VEP (Visual Evoked Potential): Consists of
presenting a reversing chess board pattern of P 100
O p tic n eu ro p ath y (L )
dark and light stimulus to the eyes from a TV L 138.00 m s 6.8 u v
monitor placed one meter away from the eyes of 25 (m s

the subject and recording the visual response Fig 8 : Visual Evoked Potential (VEP) :
from the occipital area of the brain through Normal on the right compared with abnormal
surface electrodes placed over the scalp at on the left. Note the moderate-to-marked
relevant sites. Normally it takes about 100 prolongation of the P100 latency with reduced
milliseconds (1/10 sec) for a light impulse from amplitude in optic neuropathy (L)
57
BAEP (Brainstem Auditory Evoked Potential): integrity of lower and upper brainstem auditory
In this modality click sounds are presented to each pathways respectively. This test is useful in
ear separately at different decibels and rates of assessing auditory nerve function in children and
stimulation while blocking the opposite ear with adults and in lesions such as multiple sclerosis
white noise. Recording is made from an active and stroke affecting conduction in the brainstem
electrode placed over the mastoid and referenced auditory pathways.
to the vertex with opposite mastoid acting as the BERA (Brainstem Evoked Response
ground. As a standard practice about 2000 Audiometry) is a modification of BAEP test and
averages are made to obtain a artifact-free signal is widely used in assessment of auditory function
consisting of five major peaks named waveform in high risk neonates suffering / recovered from
I to V, each waveform representing the generator hyperbilirubinaemia, hypoxic ischaemic
of the potential as auditory impulse passes encephalopathy (Fig 9), meningitis, convulsions
sequentially along the auditory nerve to the and in preterm low birth weight babies and
auditory cortex. Waveforms VI and VII which children with hearing deficiency.
are believed to be generated in the auditory cortex
are inconsistent and inconstant and therefore are SSEP (Somatosensory Evoked Potentials): The
not included in the measurement. Latency to sequential conduction of a nerve impulse through
waveform I, III and V and interpeak latencies I- the peripheral nerves (such as the median, ulnar,
III, III-V and I-V as well as amplitudes of tibial and peroneal) to the spinal cord and from
waveform I and V are measured. Auditory nerve there through the spinal somatosensory pathways,
function is represented by the latency and the brainstem and thalamic pathway to the
amplitude of waveform I. The interpeak latencies cerebral sensory cortex can be recorded and the
I-III and III-V give a measure of conduction and latency and response in terms of voltage
BA E P C lick BA E P C lick
V
NO RM AL H IE
IV
III I V 100 dB
80 dB
I II Ia III IV
1:
7R
1:
6R
Ia
1:
1L II
Va
Va 1:
2L
80 dB
1:
2R
1:
1 R
60 dB
1:
3L
4 60 dB
1:
3R
1:
4 R
40 dB
40 dB 1:
8
5R
1: R
5
1:
6L
1 (m s) 1 (m s)
Fig 9: Brainstem Evoked Response Audiometry (BERA): Left Normal response consists of
waveform V elicited at 40 dB and I to V waveforms with normal latency and amplitude
at 80 dB Right waveform V elicited only at 80 dB indicating severe hearing deficit.
This finding is supported by delayed waveform I with very low amplitude of
waveforms I to V
58
2003; 5(2):147
measured. The peripheral nerve is stimulated and helps to locate lesions in the spinal cord,
recording is made from surface electrodes placed brainstem or cerebral cortex. The test is useful
over the limbs, spine or scalp region overlying in multiple sclerosis, stroke, brainstem lesions,
the sensory cortex. This gives a measure of the spinal cord tumours, transverse myelitis etc.(Fig
sensory conduction in each of the segments and 10, 11)
M edian SE P M edian SE P
N O R M AL C E R V IC A L C O R D C O M P R E S S IO N
N 20
1:C3'
1:C3'-
-Fz
Fz:
:R
R N 20 1:C4'
1:C4'-
-Fz:
Fz:L
L
P 22
N 1 3 -N 2 0 7 .8 6
P 22 m s
N 13
N 13 2:C5s-Fz:L
N 1 3 -N 2 0 6 .1 7 m s2:C5s-Fz:R
E P -N 1 3 4 .6 1 m s
EP E P -N 1 3 4 .5 5 m s EP
3:EPi-Fz:R 3:EPi
3:EPi-
-Fz
Fz:
:L
L
5 (m s) 5 (m s)
Fig 10: Somatosenspry Evoked Potentials (SSEP) from upper limbs (median nerve
stimulation). Recording made from Erbs point, C5 spine and contralateral
scalp area (C4). EP, N13 latency normal but N13-N20 interlatency (normal
upto 7 ms) is prolonged due to cervical cord compression.
TibialSEP TibialSE P 1:
Cz'
-Fz:
L
N 45 NORM AL T ran sverse m yelitis
1:C z'-Fz:R
1:C z'-Fz:R
N 45
P 37
P37
LP
2:T12-Ic:R 2:
T12-
LP
2:T12-Ic:R T12-I
2: c:
c:L
I L
PF
3:PopF-Kn:R PF 3:
PopF-
PopF-Kn
3: Kn:
:L
L
10 (m s 10 (m s
Fig 11 : Somatosensory Evoked Potentials (SSEP) from lower limbs in transverse myelitis
compared to normal. Note the marked prolongation of the P37 latency and decreased amplitude
recorded from the scalp overlying leg area of the cortex. The N22 P37 latency is prolonged (28
ms) in transverse myelitis indicating delayed conduction in spinal somatosensory pathways
(normal 15 ms).
59
An extension of this test is dermatomal SEPs The study is useful in obstructive sleep apnoea,
in which the dermatome is stimulated and the excessive day time sleepiness due to different
conduction along the particular root subserving causes, narcolepsy (sleep attacks), restless legs
the dermatome is studied as in cervical or syndrome, sleep walking, sleep talking, nocturnal
lumbosacral radiculopathies and brachial seizures, enuresis, sleep terrors etc. Sleep centres
plexopathies in children and adults. Entrapment are very few in our country, but is an important
of the peripheral nerves such as the lateral femoral requirement in the comprehensive study of sleep
cutaneous nerve of the thigh can be studied by related medical problem in children.
stimulating the dermatomal area of the nerve and
recording from the scalp. The delay in latency Bibliography
between normal and affected side clinches the 1. Aminoff, MJ, Electrodiagnosis in Clinical Neu-
diagnosis. rology, Churchill Livingstone , New York,
2000.
Polysomnography (PSG) is a fairly recent
modality of investigation in which overnight 2. Misra UK, Kalita J. Clinical Neurophysiology,
recording (for 8-12 hours) is made of EEG BI Churchil Livingstone, New Delhi, 1999.
waveforms, respiratory and cardiac parameters
and any abnormal limb movements during sleep.
NEWS AND NOTES

PEDINEUROCON-2003
5th NATIONAL NEUROLOGICAL CONFERENCE &
31st RAJASTHAN STATE IAP CONFERENCE-2003
HOST: RAJASTHAN STATE BRANCH IAP
A three day conference is being organized on 7-9th Nov 2003 at Pink City Jaipur
Highlights: Core group meeting on development of protocols for common pediatric neurological
illnesses.
Workshops on EEG, Neuroimaging, Electromyoneurodiagnosis
Guest orations, Poster presentations, Award papers, Panel discussion
Registration Fee Up to 30 June03 31 Aug03 6 Nov03 Spot
IAP Member Rs 700 Rs 900 Rs 1200 Rs 1500
Non IAP Member Rs 800 Rs1000 Rs 1300 Rs 1600
Assoc. Member Rs 500 Rs 700 Rs 1000 Rs 1500
PG students Rs 500 Rs 700 Rs 1000 Rs 1500
Fee for each one day workshop is Rs 1, 000 BESIDES REGISTRATION
Send your demand drafts in favor of PEDINEUROCON-2003 payable at Jaipur.For
Further details please contact Dr H S Bhasin, Organizing Secretary Pedineurocon-2003
Secretariat: 476A/5 Vyas Marg, Raja Park, Jaipur 304002, Ph 0141- 2621962
Email: [email protected]
60
2003; 5(2):149
NUTRITION
SMART NUTRIENTS AND BRAIN 60 kg and brain weight is 1400 g, thus the body
DEVELOPMENT grows 20 times compared to the four fold increase
in brain growth. The interesting point is that most
* Elizabeth K E of the brain growth occurs in the first few years
of life. In the first half of infancy, brain growth
The expression of the genetic potential for
becomes 50%; by 2 years it becomes 80%, 90%
growth and intelligence is the sum total of the
by 5-6 years and almost 95% by 10 years of age.
interplay of the genetic/host factors with nutrition
By 2 years of age, body growth becomes only
and environment. Brain is the soul of the human
20% of that of the adult, but brain growth
being, the key area concerned with our
becomes almost 80%. Thus it is clear that most
intelligence, personality, emotions, well-being,
of the brain growth is directly influenced by
spirituality and probably our existence. As most
breast feeding and complementary feeding. The
of the brain growth occurs in utero and early
human brain consists of 100 billion neurons,
postnatal period, the role of maternal nutrition,
almost 1000 billion glial cells as supporting cells
breast-feeding and complementary feeding
and their connections.
cannot be underestimated. The nutrients that help
in the development of brain can be called as Two third of the weight of the brain is due
smart nutrients. to phospholipids and long chain polyunsaturated
The number of neurons is almost fixed by fatty acids (LCP). Breast milk is the best for brain
mid - gestation and it is difficult to malnourish a growth, neuromuscular development and
foetus during this period. Most of the myelination. Thus milk is now found to be
malnutrition occurs in the third trimester of species specific. Low birth weight premature
pregnancy. The migration of the neurons to the babies (LBW) fed on preterm milk secreted by
respective areas, the connections, the the mothers are found to have 10 IQ points more
proliferation of synapses, receptors and dendrites than artificially fed babies3,4. Hence milk is also
progress in the perinatal period and early baby specific.
postnatal period, especially first 2-3 years of life. The smart nutrients that promote brain
These are modifiable to a great extent through growth are variable and belong to various food
diet, specific nutrients especially micronutrients, groups. The food that we eat also influence our
stimulating environment, tender loving care memory, concentration, comprehension,
(TLC), parental interaction and play1,2. judgment, intellect, mood, emotions etc. There
The weight of a baby at birth is 3 kg and is are over 50 neurotransmitters that are affected
only 5% of the adult weight, whereas the weight by the food and the micronutrients that we take.
of the brain is 350 g, almost 25% of that of the Carbohydrate
adult brain. The average adult weight is around
* Associate Professor, Department of Pediatrics, Among the carbohydrates, lactose is credited
SAT Hospital, Medical College, as a smart nutrient. It promotes the synthesis of
Trivandrum, Kerala. cerebrosides and myelination. It is a prebiotic
61
substance that promotes lactobacilli and thereby omega 3 fatty acid, linolenic acid and ArA is
digestion. It also promotes the absorption of derived from omega 6 fatty acid and linoleic acid.
calcium and magnesium. The lactose content of These are 30 times more in breast milk than in
breast milk is double that of cows milk. cows milk. Infants are unable to convert the short
chain omega 3 fatty acid, alpha linolenic acid into
Protein DHA. The requirement of DHA is found to be
Sulphur containing amino acids are brain 20 mg/kg/day during brain growth. Fish and fish
friendly. Cysteine to methionine conversion is oils are important sources of omega3 fatty acids
low in the brain of the infant and so high and DHA. These are seen to maintain integrity
cysteine: methionine ratio in breast milk is of cell membrane in the brain. Almost half of
advantageous for CNS development. Amino the lipid in brain cell membrane is DHA. DHA
acids are the main precursors of neurotrans is the building block for cell membrane and
mitters. High tryptophan to neutral aminoacid synaptic connections. Long chain
ratio in breast milk is beneficial to the brain. polyunsaturated fats (LCP) are being tried in
Tryptophan is the precursor of serotonin. comorbid conditions like hyperactivity, attention
Serotonin is important for mood and sense of deficit disorders, dyslexia etc. Ketogenic diet is
well-being. Choline, a vital amine, is crucial for considered the final answer in intractable
brain development and is the precursor of myoclonic seizures.
acetylcholine, which is important in neurotrans Micronutrients
mission and memory. Serotonin is the feel good
neurotransmitter and acetylcholine is the These are the vitamins and minerals that are
memory-boosting chemical. Tyrosine is the required only in small quantities, but serve key
precursor of dopamine and is functional in motor functions in the body and brain. Micronutrients
coordination. Taurine is instrumental in brain are cofactors in metabolic pathways and are
growth and maturation of retina. Taurine is an essential for production of several enzymes and
important neurotransmitter and neuromodulator brain development 5,6.
of brain and retina. The low content of aromatic
amino acids like tyrosine and phenylalanine, Among the B complex factors, B1,B2,B3,
which are less utilized by small babies, also seems B6, B11 and B12 are important for the synthesis
to be protective to the brain. Moreover, the amino of various neurotransmitters. Thiamin (B1) is
acids in trans-form are brain- friendly whereas essential in the function of brain and peripheral
those in cis-form as in micro waved formula are nerves. B1 deficiency decreases the ability to
neurotoxic. utilize glucose. Deficiency is also associated with
insomnia, loss of memory, visual acuity,
Lipids Wernickes encephalopathy, Korsakoffs
psychosis and absent knee jerk. Dry beriberi is
Over 60% of the brain weight is due to known to cause neuropathy, aphonia etc.
phospholipids and long chain polyunsaturated Riboflavin (B2) deficiency is associated with
fatty acids. The derived lipids, docosahexaenoic neuromotor incoordination, personality changes
acid (DHA) and arachidonic acid (ArA) are and impaired performance in psychomotor tests.
essential for brain and neuromuscular Niacin (B3) deficiency leads to pellagra with
development. ArA is the precursor of dementia and impaired cognition. Pyridoxine
prostaglandin. DHA increases the level of (B6) is essential for the synthesis of GABA, the
serotonin and acetylcholine. DHA is derived from
62
2003; 5(2):151
inhibitory neurotransmitter and its deficiency results in deficiency of dopaminergic D2

may cause convulsions and peripheral receptors leading to reduced dopamine and
neuropathy. B6 deficiency is also associated with thereby relative increase in opiates. This shift is
sideroblastic anemia. Folic acid (B11) is essential the cause of reduced learning ability in iron
for neural tube development and periconceptional deficiency. The balance between dopamine and
supplementation of folic acid can prevent neural opiates influences learning ability. When opiates
tube defects. Folic acid deficiency is also take the lead, this ability comes down. Iron is
associated with chronic diarrhoea, attention also important in serotonin and GABA levels.
deficit disorder, stroke and autism. Folic acid Iron deficiency is also known to make the RBCs
and choline are also important for myelin rigid thereby increasing the chance of thrombosis.
synthesis. Cobalamine (B12) deficiency is Conventionally, polycythemia is said to increase
associated with subacute combined degeneration the incidence of thrombosis and stroke. Increased
of spinal cord, dementia and probably emotional iron content is also not desirable. It is connected
instability. B11 and B12 deficiency produce to Parkinsons disease in adults and Hallervorden
megaloblastic anaemia. Deficiency of folic acid Spartz disease. Similar to iron, copper is an
(B11), B12, B6 and choline leads to elevated important component of cytochrome oxidase and
levels of homocysteine that may result in also another enzyme called super oxide
thromboembolic episodes and stroke. Vitamin A dismutase. As iron and copper share several
is important in integrity of the eyes and vision. properties copper is aptly called the iron twin.
Both iron and copper deficiency are known to
Vitamin C deficiency is attributed to reduced produce microcytic, hypochromic anaemia.
resistance power, reduced IQ score, altered Copper deficiency leads to Menkes kinky hair
behaviour and increased incidence of stroke. disease and excess is associated with Wilson
Vitamin E is the potent antioxidant; its deficiency disease, Indian Childhood Cirrhosis (ICC) and
is concerned with transient ischaemic attacks probably amyotrophic lateral sclerosis and
(TIA), stroke, oxygen toxicity and probably dementias like Alzheimers. Aluminium excess
Alzheimers disease. is also suspected in the pathogenesis of
Among the minerals, iodine deficiency is the Alzheimers disease. Zinc is the constituent of
prototype of mental deficiency caused by a about 200 metalloenzymes with high activity in
nutritional deficiency. Iodine deficiency leads the brain. Zinc deficiency is associated with
to reduced physical activity, growth and diarrhoea, infertility, growth retardation and
development and is referred to as endemic neuropsychological problems. Selenium
cretinism. And iodine supplementation leads to deficiency is attributed with anxiety, oxidant
substantial improvement if undertaken early. Iron stress, depression, low mood etc. It is a powerful
deficiency is known to cause irreversible changes antioxidant. Chromium deficiency is said to
in the growing brain. It results in neuromuscular reduce glucose tolerance. Cobalt is useful in
incoordination, reduced physical activity, iodine utilization.
attention and cognition. It also leads to headache Antioxidants
and lack of concentration and impaired auditory
and visual brain stem evoked potentials. Brain is the seat of abundant metabolic
Cytochrome oxidase in mitochondria is an iron activity and hence abundant waste products.
dependent enzyme and oligodendrocytes require Brain consumes 20-30% of entire energy in spite
iron for the synthesis of myelin. Iron deficiency of having only 2% of body weight. It utilizes
63
maximum amount of glucose and oxygen. In the Apart from the specific deficiencies, protein
bargain, lot of oxygen free radicals and reactive energy malnutrition (PEM) is known to cause
oxygen species (ROS) are produced as growth arrest, arrest of milestones, regression of
byproducts. These bullets attack the cell social smile, inability to explore and master the
membrane, mitochondria and damage even the environment, irritability and apathy. PEM leads
DNA. It leads to shrinkage and dissolution of to functional isolation as in the case of hibernation
dendrites and synapses and disrupts the adapted by certain animals to tide over adverse
communication system in the brain. The content situations. This functional isolation in the child
of fat in the brain makes it vulnerable to lipid per will evoke less interaction from the mother;
oxidation by these oxidants. Antioxidants, both caretakers and peer group, resulting in poor
endogenous and dietary are the only answer to stimulation, TLC and play.
this disaster. Beta-carotene, vitamin C, vitamin
E, selenium and other phytonutrients act as Fish is aptly called a brain friendly food
scavengers and antioxidants. Copper, iron, second only to breast milk. This is now made
vitamin B complex factors are functional in the more available through the blue revolution.
endogenous antioxidant systems like glutathione Fish and fish oils are the source of the omega3
peroxidase, superoxide dismutase, catalases, fatty acids, DHA, iodine, zinc and taurine. The
ceruloplasmin etc7. so called fast food and junk food are called brain
busting food due to the deficiency of protective
Others factors and the presence of excess omega 6 trans
fatty acids which make the cell membrane of the
Non-protein nitrogens like urea, amino brain more rigid and less pliable. The omega 6
acids, peptides, nucleic acids, nucleotides, to omega 3 fatty acid ratio should be kept <5:1.
choline, creatine, creatinine, uric acid, ammonia,
polyamines, N Acetyl glutamine, N Acetyl Greens are the other sources of omega 3
neuraminic acid are bioactive factors. These fatty acids. Green leafy vegetables and green,
are seven times more in breast milk than in cows yellow orange vegetables and fruits are rich
milk. The exact role of each of these is under sources of micronutrients and antioxidants.
study. Other bioactive factors in breast milk are These are now more available through the
lactoferrin, enzymes, hormones, growth factors, rainbow revolution.
oligosaccharides, mucins and probiotic factors. Breakfast should be considered the brains
Probiotic factors help in digestion, eg, lactic acid, food. After 8 hours fast, the body reaches the
lactobacilli, bifidus factor etc. Polyamines like lowest or basal levels of nutrients and energy in
spermine, spermidine and putrescine promote cell the morning and so breakfast is like the petrol
growth and differentiation. Putrescine is the for the vehicle in reserve. Unfortunately and
precursor of GABA. Epidermal growth factor, unknowingly many children skip the breakfast
nerve growth factor, betacasomorphin, thyroxine, and start the race for the day. Mothers should
growth hormone releasing factor etc play a role take balanced diet during pregnancy and
in CNS development. Colostrum is rich in lactation. She should deliberately take extra
enzymes like lysozyme, peroxidase, xanthine nutrients for the growth and intelligence of her
oxidase which promote cell maturation. Carnitine baby. No baby should be denied the merits of
is another substance important in lipid oxidation breast feeding and optimum complementary
and various other functions. feeding during early part of life.
64
2003; 5(2):153
Reference Breast feeding and cognitive development: A

1. Carper J. Your Miracle Brain. Harper Collins Meta analysis. Am J Clin Nutr 1999; 7014:525-
Publishers. New York, 2000; pp10-19. 535.
2. Ludingtone Hoe S, Golant SK. How to have 5. Petro R Vitamins and IQ. Brit Med J 1991; 302/
smart babies? Banton Books, New York, 1985; 6781:906-908.
pp21-32. 6. Schoenthaler S. Brains and vitamins. Lancet
3. Lucas A. Breast milk and subsequent intelli- 1991; 337/8743: 728-729.
gence quotient in children born preterm. Lan- 7. Elizabeth KE, Micronutrients. In Nutrition and
cet 1992; 339:261-264. Child Development,2nd Edition, Paras Publish-
4. Anderson JW, Johnstone BM, Rembely DT. ing, 2002; pp:86-114.
NEWS AND NOTES

Indian Academy of Pediatrics Pediatric Hematology - Oncology Chapter
National Training Project Workshops in
PRACTICAL PEDIATRIC ONCOLOGY - 2003
Two day workshops in practical aspects of pediatric oncology for practising pediatricians, pediatric
surgeons and pediatric postgraduates will be held this year at - Hyderabad, Vadodara, Kolkata, Jaipur &
Bhopal (proposed). Registration is now open on first come and zonal basis. Prior registration approximately
two months before the workshop is mandatory. A reference manual of Practical Pediatric Oncology will be
provided free to the delegates about one month before the workshop. This course will help the delegates to
participate in managed shared care of pediatric cancer. For details contact the national co-ordinator or trhe
respective workshop co-ordinators:
NATIONAL CO-ORDINATOR:
Dr. Bharat R. Agarwal
Head of Department, Division of Pediatric Hematology - Oncology, B.J. Wadia Hospital for Children,
Parel, Mumbai - 400 012. Telefax : 00-91-22-26431902, 26426846 Email: [email protected]
Workshop at Hyderabad: on 22nd & 23rd August 2003:
D. Raghunadharao, MD DM
Professor and Head, Department of Medical Oncology, Room Number #607, 6th Floor, E Block
Nizams Institute of Medical Sciences, Panjagutta, Hyderabad 500 082. Andhra Pradesh
Tel: 040 23371747, Fax: 040 55669049, Mobile: 040 56871537
Emails: [email protected] and [email protected]
Workshop at Bhopal: dates to be finalised
Dr. Shyam Agarwal
Navodaya Oncological Research Center, 108, Zone II, M.P. Nagar, Near Bhopal Eye Hospital,
Bhopal - 462 011.M.P. Tel. No. Off. 272220-19, Res: 551488, Mobile: 98270-55790
Workshop at Kolkata:
Dr. Ashish Mukhopadhyay
Ideal Tower, Flat 7C & 8, 57, D.H. Road, Kolkata - 700 023. Tel.No.Off. 4567050-59, Res: 4486362
Email: [email protected] / [email protected]
Workshop at Jaipur: Dr. Rajiv Kumar Bansal, 12, Rathapuri, Sodala, Ajmer Road, Jaipur - 302 006. Rajasthan
Workshop at Vadodara:
Dr. Vibha Naik
Dr. Naik Hospital, Kasar Phadia, Opp.Govt. Press, Kotti, Baroda, Gujarat, Tel.No. 0265-2412311, 2434788
Res: 0265-2392149, 2393538, Mobile 98250-29085
65
IJPP-IAP CME
RECOGNITION OF A CRITICALLY is important to identify a child with physiological

ILL CHILD derangement in its early stages when signs are
subtle. The golden hour concept applies to all
* Ramachandran P children with illnesses presenting as emergency.
How to identify a critically ill child? Early recognition of a critically ill child requires
a systematic and rapid clinical assessment with a
What are the predisposing conditions? background knowledge of age appropriate
What is the initial management of critically physical signs.
ill child?
There are many methods to assess an acutely
Critically ill child means a child who is in ill child. A very simple and quick way of
a clinical state which may result in respiratory or assessment of overall illness and injury severity
cardiac arrest or severe neurologic complication, is by:
if not recognised and treated promptly. This term
does not refer to any particular disease, but many 1. Appearance of the child
diseases can lead onto critically ill state. 2. Breathing
Whether a child presents with a primary
cardiovascular, respiratory, neurologic, infectious 3. Circulatory status
or metabolic disorder, the goal is early These three parameters comprise Pediatric
recognition of respiratory and circulatory assessment triangle
insufficiency. In clinical practice, there are three Appearance
common situations that characterise a critically
ill child:
1. Respiratory distress
2. Shock Breathing Circulation
3. Altered sensorium .
Appearance of the child
Early intervention is geared towards Appearance basically denotes the
preventing the progression of hypoxemia and neurological status. It is determined by the
hypoperfusion to full cardiorespiratory arrest. oxygen and blood supply to the brain which are
It is easy to recognise a critically ill child dependent on cardiopulmonary status and the
when there is an obvious problem like severe structural integrity of the brain. The parameters
trauma or unconsciousness. On the other hand it assessed in appearance are alertness,
distractibility or consolability, eye contact, speech
* Asst. Prof. of Pediatrics, or cry, motor activity and color of the skin. In
Pediatric Intensive Care Unit, addition, seizures, abnormal posturing, muscle
Institute of Child Health and Hospital for
tone and pupillary reaction are noted.
Children, Chennai - 8.
66
2003; 5(2):155
1. Alertness: Normal children exhibit Breathing

awareness and interest in surroundings.
Determine if the child is confused, irritable, 1. Respiratory rate: Tachypnoea is an early sign
lethargic or totally unaware of environment. of respiratory distress. Tachypnoea without
Changes in level of conscious can also be increased work of breathing (Quiet
rapidly assessed by AVPU method. tachypnoea) is seen in shock, heart disease
and acidosis. A slow or irregular respiratory
Awake rate in an acutely ill child is ominous.
Responsive to voice
2. Work of breathing: Increased work of
Responsive to pain breathing (IWB) indicates respiratory
Unresponsive distress or potential respiratory failure. IWB
is assessed by nasal flaring, grunting,
2. Distractibility or consolability by parent is a
intercostal, subcostal and suprasternal
normal phenomenon in infants and young
retractions. Head bobbing and see saw
children.
respirations (severe chest retraction with
3. Eye contact with parents or physician is abdominal distension) are more advanced
noted normally at 2 months of age. Failure signs of respiratory distress and impending
to do this, is an early ominous sign of cortical respiratory failure.
hypoperfusion and brain dysfunction.
3. Air entry: Effective tidal volume is assessed
4. Speech / cry: Whether the cry is normal or by chest expansion and auscultation of breath
whimpering or moaning or high pitched sounds.
5. Motor activity: Normal movements of limb, 4. Pulse oximetry: Oxygen saturation
trunk and neck. assessment is an important adjunct to identify
6. Colour of the skin: denotes respiratory and oxygenation state in acutely ill child.
circulatory status. Skin of palm and fingers Circulatory Status
may be pink (normal), pale, cyanosed,
mottled or ashen grey. Circulation is assessed to find out if the
cardiac output meets the tissue demand. Shock
Other features in appearance are: is defined as circulatory dysfunction in which
Seizure activity: Seizures with altered there is inadequate delivery of oxygen and
sensorium is a critically ill state as it may lead substrates to meet the metabolic demands of
onto cardiorespiratory compromise or neurologic tissues. Circulatory status is assessed by heart
sequalae if not treated. rate, skin perfusion, systemic perfusion and blood
pressure.
Posturing: Intermittent flexor (decorticate)
or extensor (decerebrate) posturing occur with 1. Heart rate: Tachycardia is a common
prolonged cerebral hypoperfusion. response to a variety of stresses including
Muscle tone: Hypotonic limp child is a bad shock. Hence its presence mandates further
sign. evaluation. Bradycardia in a critically ill
child is ominous
Pupil size: Pupils may be small but reactive
in cerebral hypoperfusion. Unequal pupils is a 2. Pulses: Comparison of central (femoral,
medical emergency; may indicate ICP. carotid and brachial) and peripheral (radial,
67
dorsalis pedis and posterior tibial) pulse Based on the appearance, breathing and
volume. In early shock peripheral pulses are circulatory status, physiologic status of a critically
weak. Loss of central pulse is a premorbid ill child is characterised as:
sign and is to be treated as cardiac arrest.
1. Stable
3. Skin perfusion
2. Respiratory distress characterised by IWB
a. Temperature: When ambient
3. Respiratory failure characterised by
temperature is warm, hands and feet
cyanosis, altered sensorium, poor muscle
should be warm. Hands and feet
tone and poor respiratory efforts.
become cold when cardiac output falls.
4. Early shock Peripheral signs with normal
b. Colour: Normally the colour is pink BP
over the palms upto the fingers. Pallor
or cyanosis or mottling denote 5. Decompensated shock Peripheral signs
decreased perfusion. with brain dysfunction and hypotension
c. Capillary refill time (CRT): Normal is 6. Cardiorespiratory failure Shock +

less than 2 seconds. Delayed CRT is respiratory failure
again a feature of early shock. Conditions characterising a critically ill child
requiring rapid cardio pulmonary assessment
4. Organ perfusion
1. Tachypnoea Respiratory rate > 60 in
a. Brain perfusion: Brain perfusion can be
newborn
assessed by features already described
in appearance i.e. changes in level of > 50 in infants
consciousness, pupil size, muscle tone > 40 in 1-5 years
and posturing.
2. Bradycardia or Tachycardia
b. Renal perfusion: Urine output may not
Newborn < 80 and > 200
be useful in initial assessment in a
critically ill child, but is useful in 1 month - 8 years <80 and > 180
monitoring the child and evaluation of > 8 years <60 and > 160
renal perfusion. 1-2 ml/kg/hour of
3. IWB and decreased tidal volume
urine output is normal
4. Cyanosis
5. Blood pressure: Shock can be present with
normal, increased or decreased blood 5. Altered level of consciousness
pressure. In early compensated shock, BP 6. Seizures
is normal. In late or decompensated shock
7. Fever with bleeding
there is hypotension.
8. Fever > 41C in less then 3 months or fever
Lower limit (5 th percentile) of Blood in immuno compromised child.
pressure
9. Trauma
Newborn 60 mm Hg systolic
Upto 1 year 70 mm Hg systolic 10. Burns totaling > 10% body surface area
2 years and above 70 + (2 x Age in years) 11. G1 bleeding
mm Hg 12. Poisoning
68
2003; 5(2):157
Initial Management of a critically ill child Common predisposing factors for a child
becoming critically ill:
When a child is assessed to be critically ill,
initial management comprises of taking care of 1. Age: Younger the age, more the risk
airway, breathing and circulation. Recognition
2. Malnutrition / impaired immune status
of acuity of illness determines the urgency of
intervention. Aggression in response is driven 3. Underlying anatomic / functional defect
by degree of physiologic compromise. 4. Nature of illness
1. Airway is kept patent if necessary by 5. Bad child rearing / traditional practices
positioning, suctioning and intubation.
6. Type of medical care received
2. Breathing: In respiratory distress with 7. Parents knowledge / awareness
increased WOB, only supplemental O2 is
given in a nonthreatening manner. Preparedness to manage critically ill child
If respiratory failure is present, ventilation 1. Oxygen source

is done with maximal supplementary 2. Oxygen delivery system -
oxygen. Oxygen mask, (infant, child size)
3. Vascular access and volume expansion with - Nasal cannula
isotonic fluids such as RL or NS. 3. Suction apparatus / suction catheters / mucus
4. Avoid oral feeds in a critically ill child. sucker (De Lee)
4. IV cannula 20, 22, 24 size
5. If partial airway obstruction is suspected,
allow the child to assume position of comfort Scalp vein needles 21, 22, 23, 24 size
and avoid any painful procedure. 5. Intraosseous needles 15 and 18 G
6. If child presents with seizures, take care of 6. Fluids: Normal saline, lactated Ringer, 25%
airway and breathing and control seizures Dextrose
with IV diazepam or lorazepam. In office
7. IV sets
practice, IM midazolam 0.15 mg/kg is safe,
effective and fast. 8. Self inflating bag (500 ml, 750 ml), valve,
mask with O2 reservoir
7. In polytrauma or head trauma open and
maintain airway with cervical spine 9. Drugs: Diazepam, Lorazepam,
stabilisation carry out volume expansion Hydrocortisone, Dexamethasone,
with early administration of blood and Inj Adrenaline 1:1000, Midazolam injections
control bleeding.
10. Nebuliser solution: Salbutamol, Ipratropium
8. In acute severe asthma, nebulisation with 11. Laryngoscope, blades
salbutamol and if necessary ipratropium.
12. Spine board
9. Plan for transport to a centre where child can
be managed further. 13. Drug dosage chart
69
Dos and donts in management of critically ill Donts

child 1. Dont fail to monitor periodically
2. Dont give only IV fluids without taking care
Dos of airway and breathing
1. Be aware of age specific emergencies 3. Dont give drugs by inappropriate route
4. Dont panic.
2. Know about current epidemic in your area
Suggested reading
3. Keep emergency drugs ready and 1. Pediatric Advanced Life Support Guidelines
resuscitation equipment in good condition 1997
American Heart Association and American
4. List the nearest hospital for referral, Academy of Pediatrics.
telephone numbers of hospitals and
2. Pediatric Emergency Medicine Course guide-
ambulance lines, Chennai 2001.
5. Inform parents about the problem and what 3. Pediatric Critical Care, 2nd Edn., Eds Fuhrman
is being done BP, Zimmerman JJ, St.Louis, Mosby 1998
NEWS AND NOTES
VII RAJNEOCON - 2003 - KOTA

Date : 20-221, September, 2003
Host : IAP Hadoti Branch Kota & NNF Raj State Chapter
The IAP Hadoti branch is organising VII Rajasthan Neonatology Conference on 20-21st September, 2003. at IMA
House, M/B.S. Hospital Campus, Nayapra, Kota. The theme is Better-Newborn care- Need of the Millenium.
Highlights : Faculty includes eminent neonatologists and intensivists of India.
Programme : Common neonatal problems with practical approach protocols and recent advances in form of guest
lectures, Panel discussion and NALS workshop.
Topics : Persistent hyperbilirubinemia. Newer guidelines on neonatal resuscitation, Mec asp. Syndrome-newer
management stratigies, neonatal sepsis, Neuroprotection in HTE, refractory neonatal sizures and fluid-electrolyte
balance in NICU etc.
Registration fee upto 31-08-03 from 1-9-03 Spot
IAP/NNF member 300 400 500
Non member 400 500 600
Accompanying member 200 300 400
NALS workshop 400 500
(Payment through, demand draft only in favour of VII Rajneocon, 2003 Kota)
Correspondence :
Dr. C.B. Das Gupta, Organising Chairperson, 1, Gulab Bari, Arya Samaj Road, Kota - 324 006.
Phone : 0744-2381723, 2322703 Email - [email protected]
Dr. Ashok Sharda, Organising Secretary, Sharda Children Hospital, 4-B-14, Talwandi, Kota - 324 005.
Phone : 0744-2428393, 2420066(H), 2426088(R) Email - [email protected]
70
2003; 5(2):159
RADIOLOGIST TALKS TO YOU
OBSTRUCTION IN URINARY Sometimes you may see the dilated upper

TRACT moiety in a duplex system as in Fig 3. When
you see double moiety, look for associated
* Vijayalakshmi G abnormalities of ureters like a ureterocele or
* Natarajan B ectopic ureter. If the ureter is also dilated then
** Ramalingam A one should expect a block at the appropriate level.
Fig 4 shows a ureter that is dilated. Look for the
In this issue we will discuss problems
cause. The commonest is a calculus. Sometimes
pertaining to dialatation of the urinary collecting
the dilation may extend upto the vesico-ureteric
system. Dilatation of the collecting system
junction. This happens in conditions like a
usually means a block somewhere along the
ureterocele or primary megaureter. The
pathway of flow. This basic inference solves
ureterocele is seen as a rounded, smooth, black
many clinical problems like urinary tract
structure projecting into the bladder (Fig. 5).
infection, a palpable kidney or colic. The
commonest obstruction that is now increasingly In primary megaureter the distal part of the
been diagnosed because of the widespread use ureter is very narrow and shows frequent
of antenatal ultrasound is pelvi-ureteric junction peristalsis. The diagnosis is confirmed with IVU.
obstruction. All structural abnormalities need to be subjected
to IVU before surgery. If there is bilateral
Fig 1 Shows a dilated pelvicalyceal system
ureterohydronephrosis it is rightly lower urinary
(PCS) consisting of round, black or cystic pelvis
tract obstruction. The important condition that
and calyces. The ureter is not dilated. So it tells
one has to be alert to is the posterior urethral
you the level of obstruction is at the PUJ. Now
valves. In this the posterior urethra is dilated
look at Fig 2. Is this a dilated pelvicalyceal
(Fig. 6).
system? Here also you see a number of cystic
spaces like the dilated calyces of a hydronephrotic This structure can be imaged from the
kidney. But this is a multicystic kidney. In this transabdominal position and perineal route. A
condition the kidney shows a number of cysts. large ureterocele, a vesical diverticulum or a
Unlike PUJ obstruction these do not presacral mass can cause bladder outlet
communicate with each other. In PUJ obstruction obstruction as also, a urethral calculus. So you
there is a central large, cystic pelvis and see that ultrasound is the first investigation in
peripherally situated smaller calyces which all suspected urinary tract obstruction.
communicate with the pelvis.
* Asst. Professor in Radiology
** Addl. Professor in Radiology
Department of Radiology
Institute of Child Health & Hospital for Children,
Egmore, Chennai.
71
Fig 1. PUJ obstruction
Fig 2. Multicystic kidney

72
2003; 5(2):161
Fig 3. Double moiety U- dilated upper moiety L lower
Fig 4. Dilated PCS and ureter (RU)

73
Fig 5. Ureterocele
Fig 6. PUV
74
2003; 5(2):163
CASE STUDY
DENGUE HAEMORRHAGIC tarry stools and then developed drowsiness and

FEVER IN A BOY WITH BOMBAY cold extremities. On examination, he was in
BLOOD GROUP A RARE shock as evidenced by absent peripheral pulses,
COINCIDENCE cold and clammy skin, unrecordable BP, and a
prolonged capillary refill time. Liver was
*Janani Shankar enlarged 4 cm below the right costal margin.
**Adhisivam B Melena was the only bleeding manifestation.
Examination of the other systems was normal.
Dengue fever is an acute febrile viral disease A diagnosis of dengue shock syndrome was made
frequently presenting with headache, bone or and he was immediately given appropriate IV
joint and muscular pains, rash and leucopenia as fluids and oxygen by mask.
symptoms. Dengue haemorrhagic fever is
characterized by four major clinical His lab investigations revealed
manifestations : high grade fever, haemorrhagic thrombocytopenia (Platelet 45,000 / cu.mm),
phenomena, often with hepatomegaly and in hemoconcentration (PCV : 42%), raised liver
severe cases, signs of circulatory failure1. Some enzymes (SGOT : 524 IU/L, SGPT : 304 IU/L),
of these children with significant clinical bleeding prolonged APTT and serology for dengue both
may require transfusion of blood or blood IgM and IgG were positive. Even with the use
products as an emergency. Sometimes a pre- of adequate volume crystalloids for 3 hours, he
existing condition of the child may interfere with was still in shock and was losing excessive blood
the management of the current problem. We from the body in the form of melena. Hence it
present here one such child with Bombay blood was decided to transfuse him with fresh whole
group who developed dengue haemorrhagic fever blood and a blood sample for cross matching was
a rare coincidence not reported in literature so sent.
far.
In the blood bank, a forward typing showed
Case report the blood group as O. However there was no
agglutination with anti H serum and a back
A four year old boy from Chennai born to typing confirmed the blood group to be of the
non consanguinous parents was brought to the Bombay phenotype (Oh). Being a rare blood
emergency room with history of high grade group, finding a suitable donor was very difficult.
continuous fever with severe myalgia for two Fortunately, a close relative of the boy was
days following which he started passing black identified to have Bombay blood group and his
blood was transfused to the patient with no
* Senior Consultant, reactions. After the transfusion he improved
hemodynamically and was discharged after a
** PG Student,
week of hospitalization.
Nungambakkam, Chennai.
75
Discussion antigen) blood to Bombay group (without H

antigen) may occur, as the Anti H in the Oh
The Bombay phenotype (Oh) is a rare blood individuals is a very potent hemolysin. This
group. Though it was first reported by Dr.Bhende will occur especially in situations in which
and Bhatia from Bombay in 1952, it is also found blood is issued without crossmatching, as in
in Caucasians. In India, it occurs with a frequency emergencies.
of 1 in 7,600 and a high level of consanguinity
has been observed among the parents of Bombay 2. The exact incidence of the Bombay blood
phenotype. The cause of this group is group which is more prevalent in India will
predominantly a mutation in the H gene on not be known and hence availability of blood
chromosome 19 that causes a non functional H donors of this rare group may not be
glycosyl transferase. Individuals with this group identified. In our hospital which is an
fail to express A, B or H antigens on their red exclusive pediatric hospital, over the past
cells or other tissues and hence no agglutination five years we have had three Bombay groups
is noted with anti A, anti B and anti H typing out of about 18,000 blood groupings done
sera. These individuals have all three antibodies for patients and two Bombay group donors
anti A, anti B and anti H in their serum 2. though out of 4,500 blood donors.
in front typing they appear as O blood group, In the above case, had the agglutination test
the presence of anti H in their serum (a potent not been done with anti H and the back typing
hemolysin of H positive red cells) makes their the patients blood would have been mistaken for
blood incompatible with O blood group O group and transfusion with that group could
individuals. Hence a patient with Bombay blood have been disastrous.
group should be transfused only with the same
blood group 3. Acknowledgement
Blood grouping should be done routinely by We herewith acknowledge the work of all
both front typing (cell grouping) and back typing blood bank technicians of Kanchi Kamakoti
(serum typing). The serum grouping serves as a CHILDS Trust Hospital and the kind donor
recheck for the front typing, as proper ABO without whose blood we would have lost the
grouping is vital in the prevention of disastrous patient.
hemolytic transfusion reactions. It is also
References
important to use Anti H lectin in front typing of
blood for the presence of H antigen in cells giving 1. WHO manual Dengue haemorrhagic fever
the reaction of O group. Usage of Anti H lectin diagnosis, treatment, prevention and control
in front typing and meticulous back typing with 2nd edn. Published by Ashok Ghose, Prentace
known A,B,O cells and serum of the individual Hall of India, New Delhi,1997, p1.
is the only way in which patients or potential 2. Frances K Widmann, Technical manual of the
blood donors with Bombay blood group (Oh) can American Association of Blood Banks, 9th edn.
be identified. Sadly, this is not done routinely in published by American association of Blood
many laboratories. banks, Arlington,Virginia, 1985, pp 177-120.
3. Mollison PL, Engelfriet CP, Marcela
The implications are
Contreras. Blood transfusion in clinical medi-
1. Hemolytic transfusion reactions due to cine 9th edn.1993, p154.
transfusion of normal O group (with H
76
2003; 5(2):165
CASE STUDY
CAFFEYS DISEASE (INFANTILE Based on the above picture the child was
CORTICAL HYPEROSTOSIS) diagnosed as having acute osteomyelitis and
was treated for around 1 month period, but there
* Mohammed Thamby was no response. The child developed same type
* Nagarajan M of swelling with discolouration over both sides
** Ram Saravanan R of cheeks. Radiology also revealed the same
features over the mandibles. It made the
Caffeys disease is a very rare pediatric pediatricians to suspect the diagnosis of Caffeys
orthopedic problem of infantile age group. It is disease.
spontaneous, self-limiting and with no definite
etiology. It has no sex or racial predilection. DISCUSSION
Report In this type of clinical presentations,

infantile cortical hyperostosis is usually kept
A female child, third of three siblings, last in the list of differential diagnosis like acute
presented at 2 months of age with swelling and osteomyelitis, hypervitaminosis A, scurvy,
discolouration of skin of short duration (few days) syphilis and trauma, since it is rare and all other
over the upper half of left tibial region. The child conditions need immediate intervention. In acute
was reluctant to move the limb and there was osteomyelitis radiological changes occur only if
tenderness on palpation. The child was highly they are left untreated for 2-3 weeks and it usually
irritable with mild to moderate fever and other involves the medullary cavity first, then
systems were normal. The child had absolutely progresses to the cortex and periosteum ,whereas
normal birth history and no trauma since birth. in Caffeys disease the pattern of involvement is
Other siblings were normal. reverse.
Blood counts of the child showed, elevated Infantile cortical hyperostosis is a self-
TC (22500 cells / cu.mm ), elevated ESR ( hour limiting disease of early infancy, characterised
- 55mm, 1 hour - 97mm), positive CRP (6 mg/L) by swelling of soft tissue, cortical thickening of
and negative VDRL test. Radiological underlying bone and hyperirritability.It has no
investigations revealed abnormal cortical definite etiology. Inherited defect of the arterioles
thickening confined to diaphysis of tibia in the of the periosteum, allergy and infective theories
upper part with coarse elevated periosteum. have been postulated in the cousation of caffeys
USG - reported periosteal thickening. CT Scan disease.
showed abnormal periosteal elevation. Usual age of onset is ninth week of postnatal
life, but cases have been reported even at birth
and as early as twenty fourth week of intra uterine
* Senior consultants
life. It starts with sudden swelling which is deep
** Junior Resident in Pediatrics
and firm with mild to moderate fever and
Child Care Centre, Tirunelveli
77
Fig 1 and Fig 2. Radiogram shows marked involvement of tibia with cortical hyperostosis and
deep soft-tissue swelling.
Fig 3. Arrowmark of the Radiogram shows Fig 4. Arrowmark of the CT scan picture of
involvement of mandible both sides. leg shows marked involvement of tibia with
cortical hyperostosis.
hyperirritability; commonly involves mandible phosphatase and positive CRP test have been
and then ulna, tibia, clavicle, scapula and ribs reported. Sometimes child may report with
are involved. Neither phalanges nor vertebrae anemia. But culture will not grow any infectious
are involved which differentiates it from agent.
hypervitaminosis A.
X-ray usually shows periosteal hyperostosis There is no specific treatment, complete
confined to diaphysis of long bones. In due course resolution in 6 9 months is the rule.
it becomes homogenous with the underlying Spontaneous remission and exacerbation may
cortex(Fig 1,Fig 2,Fig 3 & Fig 4). It takes several occur. Corticosteroids are effective in alleviating
months and even a year to resolve. Laboratory acute symptoms but has no role on reverting bony
values like elevated ESR, elevated alkaline changes.
78
2003; 5(2):167
CASE STUDY
NEUROFIBROMATOSIS TYPE I with no signs of union along with sclerosis and

WITH CONGENITAL obliteration of medullary cavity. Orthopaedic
PSEUDOARTHROSIS AND consultation confirmed that this was the typical
presentation of congenital pseudoarthorosis.
HOLOPROSENCEPHALY
There was no positive family history.
*Preetha Prasannan Since admission, the infant was getting
**Veerendra Kumar myoclonic seizures of increasing severity. EEG
***Jayakumar showed a hypsrrhythmia pattern and CT scan
***Sukumaran TU revealed holoprosencephaly. The infant was put
Neurofibromatosis type I (NF-I) is a neuro on temporary bracing of the leg to prevent further
cutaneous syndrome with diagnostic criteria displacement of ununited fracture segments and
including distinct cutaneous and osseous lesions1. is awaiting corrective surgery. Seizures were
This includes congenital pseudoarthrosis of tibia, controlled with high doses of sodium valproate
a rare anomaly with incidence of one per two and prednisolone.
lakh live births2 and which when present is a Discussion
strong pointer to NFI. Here we present an infant
Neurofibromatosis type I (Von
with NFI with congenital pseudoarthrosis who
Recklinghausen disease) is an autosomal
on further evaluation had a developemental
dominant disorder which results from an
malformation of brain, holoprosencephaly.
abnormality of neural crest differentiation and
A five month old female baby presented with migration during early stages of embryogenesis.
multiple caf au lait spots, more than six in
number with greatest transverse diameter more
than five millimeters. She also had a swelling in
lower end of right leg noticed since three
months.On examination, there was anterolateral
bowing of lower leg with abnormal mobility,
simulating a joint, being elicited at the junction
of middle and distal thirds of tibia and fibula.
X-rays taken three months back and presently
on admission revealed a fracture at the junction
of middle and distal thirds of tibia and fibula,
* PG Student
** Lecturer
*** Assistant Professor
Institute of Child Health,Medical College, Fig. 1. Neurofibromatosis type I with cafe-au-
Kottayam, Kerala. lait spots and congenital pseuarthrosis MBIA
79
First clinical and pathological account of the

disease was given by Von Recklinghausen in
1882. Tilesius gave the first description of a
patient with multiple fibrous skin tumors in 1793.
Incidence is 1/4000 and is diagnosed if two out
of the following are present1.
1. Six or more caf au lait spots of more than 5
mm. transverse diameter which may be
present at birth. They are present in almost
100% of NF- I.
2. Axillary or inguinal freckling manifest
around puberty.
3. Two or more Lisch nodules - These iris
hamartomas increase with age, incidence Fig. 2. Neurofibromatosis type II with cafe-
being 5% below three years to 100% above au-lait spots and congenital pseuarthrosis
21 years. MBIA
4. Two or more neurofibromas or one
plexiform neuro fibromatosis. Neurofibro-
mas are small rubbery lesions which appear
around adolescence. Plexiform neuro
fibromatosis involves diffuse thickening of
nerve trunks which may produce overgrowth
and deformity of an extremity. This may be
present at birth.
5. A distinct osseous lesion such as sphenoid
dysplasia or congenital pseudoarthrosis tibia.
6. Optic nerve gliomas in around 15% which
give rise to an afferent pupillary defect. Fig. 3. Pseudoarthrosis R Tibia and Fibula
7. A first degree relative with NF-I. 50% of inneurofibromatosis
NF- I are inherited, rest result from sporadic
mutations. constriction of tibia present at birth. Spontaneous
As 2 out of 7 diagnostic criteria are satisfied fracture or fracture following minor trauma
in this baby, a diagnosis of NF I was made. occurs before two years. In the other types, there
Congenital pseudoarthrosis of tibia, is a may be a congenital cyst, sclerotic segment,
specific type of nonunion, that at birth may be dysplasia or intra-osseous neuro fibroma which
present or incipient. Incidence is one in two lakh fractures and heals poorly. Treatment consists of
live births2. Cause is unknown, but occurs almost early bone grafting and intra-medullary fixation.
always with NF-I, the favoured site being distal Congenital pseudoarthrosis responds poorly to
third of tibia and fibula. Boyds classification treatment with multiple failed surgical procedures
includes six types, the commonest being type and repeated fractures which may necessitate
II which is due to anterior bowing and hourglass amputation.
80
2003; 5(2):169
Fig. 4. Congenital Pseudoarthrosis R Tibia Fig. 5. Holoprosencephaly in neurofibro-

and Fibula matosis I
NF I patients are susceptible to References

neurological complications including neuronal
1. Robert HA, Haslam. Neuro cutaneous syn-
migration disorders and cerebral heterotopias. dromes, In:Nelson Text Book of Pediatrics, 16th
Various brain tumours including astrocytomas, edn, eds, Behrman, Kleigman, Jenson, WB
meningiomas, optic gliomas, schwannomas and Saunders company, Philadelphia, 2000; pp
cerebral vessel thrombosis, stenosis and 1835 1836.
hydrocephalus have been described. 2. James H, Beaty. Congenital anomalies of lower
extremity. In: Campbells operative ortho-
This infant had seizures with
paedics-Volume-I, 9th edn, eds S. Terry Canale,
holoprosencephaly being detected on CT scan Mosby - A Times Mirror Company, St. Louis
brain. It is a developemental defect of brain which Missouri, 1998; pp 957-961.
results from defective cleavage of the 3. Bruce O, Berg. Neuro cutaneous syndromes -
prosencephalon 3,4. It is characterized by a phakamatoses and allied conditions. In: Pedi-
globular brain with absence of inter hemispheric atric Neurology - Principles and Practice (Ken-
fissure, absent falx, fused ventricles, fused basal neth F Swaiman) 3rd Edn volume I, eds, Ken-
ganglia. Incidence ranges from 1/5000 to 1/ neth F, Swaiman, Stephen Ashwal, Mosby, St.
16000. Cause is unknown in majority of cases, Louis, Missouri, 1999; pp 530-533.
with certain chromosomal aberrations accounting 4. Stephen Ashwal. Congenital structural defects.
for a minority. Affected infants die during In: Pediatric Neurology - Principles and Prac-
infancy. tice ( Kenneth F Swaiman) 3rd Edn volume I,
eds Kenneth F, Swaiman, Stephen Ashwal -
As there is no definite treatment for NF, Mosby, St. Louis, Missouri,1999; pp 251-254.
supportive treatment in the form of correction of 5. Srikant Basu and Rasmi Sarkar. Neuro fibro-
pseudoarthrosis and control of seizures can be matosis type I in a family. Images in clinical
offered to this infant5. But the ultimate prognosis practice. Indian Pediatrics, 2001;38(6): pp
appears grim due to the associated brain anomaly. 670.
81
GLOBAL CONCERN
SEVERE ACUTE RESPIRATORY directly when droplets are inhaled by another

SYNDROME (SARS) person, or indirectly when droplets land on an
object or surface (such as a doorknob or
Introduction: telephone) that are then touched by another
Severe Acute Respiratory Syndrome individual.
(SARS) is an acute respiratory illness that has Information to date suggests that people are
recently been reported in Asia, North America, most likely to be infectious when they have
and Europe. The majority of patients identified symptoms, such as fever or cough. However, it
as having SARS have been adults aged 25 - 70 is not known how long before or after their
years who were previously healthy. Few symptoms begin that patients with SARS might
suspected cases of SARS have been reported be able to transmit the disease to others. Cases
among children aged <15 years. of SARS continue to be reported primarily among
Agent suspected to cause SARS: people who have had direct close contact with
an infected person, such as those sharing a
Scientists at CDC and other laboratories
household with a SARS patient and health-care
have detected a previously unrecognized
workers who did not use infection control
coronavirus in patients with SARS. Genetic
procedures while caring for a SARS patient.
analysis suggests that this new virus belongs to
the family of coronaviruses but differs from Clinical features:
previously identified coronaviruses .While the
The incubation period for SARS is typically
new coronavirus is still the leading hypothesis
2-7 days; however, isolated reports have
for the cause of SARS, other viruses are still
suggested an incubation period as long as 10 days.
under investigation as potential causes.
The illness begins generally with a prodrome of
Spread of SARS: fever (>100.4F [>38.0C]). Fever often is high,
The principal way SARS appears to spread sometimes is associated with chills and rigors,
is through droplet transmission; namely, when and might be accompanied by other symptoms,
someone sick with SARS coughs or sneezes including headache, malaise, and myalgia. At the
droplets into the air and someone else breathes onset of illness, some persons have mild
them in. It is possible that SARS can be respiratory symptoms. Typically, rash and
transmitted more broadly through the air or from neurologic or gastrointestinal findings are absent;
objects that have become contaminated. however, some patients have reported diarrhea
during the febrile prodrome.
Droplet transmission refers to the spread of
viruses contained in relatively large respiratory After 3-7 days, a lower respiratory phase
droplets that people project when they cough or begins with the onset of a dry, nonproductive
sneeze. Because of their large size, droplets travel cough or dyspnea, which might be accompanied
only a short distance (usually 3 feet or less) before by or progress to hypoxemia. In 10%20% of
they settle. Droplet transmission can occur either cases, the respiratory illness is severe enough to
82
2003; 5(2):171
require intubation and mechanical ventilation. of patients, the respiratory phase is characterized
The case-fatality rate among persons with illness by early focal interstitial infiltrates progressing
meeting the current WHO case definition of to more generalized, patchy, interstitial infiltrates.
SARS is approximately 3%. Some chest radiographs from patients in the late
The severity of illness might be highly stages of SARS also have shown areas of
variable, ranging from mild illness to death. consolidation.
Although a few close contacts of patients with Serum antibody tests, including both
SARS have developed a similar illness, the enzyme immunoassay (EIA) and indirect
majority have remained well. Some close contacts immunofluorescence antibody (IFA) formats,
have reported a mild, febrile illness without have been developed. A positive test result means
respiratory signs or symptoms, suggesting the that both types of antibody tests were used and
illness might not always progress to the that results for both were positive. At this time,
respiratory phase. CDC is interpreting positive test results to
indicate previous infection with this newly
Investigations:
recognized human coronavirus. However, some
Early in the course of disease, the absolute people do not test positive until more than 21
lymphocyte count is often decreased. Overall days after onset of illness.
white blood cell counts have generally been
Reverse transcription-polymerase chain
normal or decreased. At the peak of the
reaction (RT-PCR) testing is also available. This
respiratory illness, approximately 50% of patients
test can detect coronavirus RNA in clinical
have leukopenia and thrombocytopenia or low-
specimens, including serum, stool, and nasal
normal platelet counts (50,000150,000/L).
secretions.
Early in the respiratory phase, elevated creatine
phosphokinase levels (as high as 3,000 IU/L) and Viral isolation for the new coronavirus also
hepatic transaminases (two to six times the upper has been done. In these studies, clinical
limits of normal) have been noted. In the majority specimens from SARS patients are co-cultured
of patients, renal function has remained normal. with well-characterized cell lines and then
laboratorians look for evidence of coronavirus
Initial diagnostic testing should include chest replication in these cultured cells.
radiograph, pulse oximetry, blood cultures,
sputum Grams stain and culture, and testing for Several laboratories have reported positive test
viral respiratory pathogens, notably influenza A results for human metapneumovirus in patients
and B and respiratory syncytial virus. Clinicians with SARS. There is not enough information to
should save any available clinical specimens determine what role, if any, human
(respiratory, blood, and serum) for additional metapneumovirus might have in causing SARS.
testing until a specific diagnosis is made. Management:
Clinicians should evaluate persons meeting the Treatment regimens have included several
above description and, if indicated, admit them antibiotics to presumptively treat known bacterial
to the hospital. Close contacts and healthcare agents of atypical pneumonia. In several
workers should seek medical care for symptoms locations, therapy also has included antiviral
of respiratory illness. agents such as oseltamivir or ribavirin. Steroids
Chest radiographs might be normal during have also been administered orally or
the febrile prodrome and throughout the course intravenously to patients in combination with
of illness. However, in a substantial proportion ribavirin and other antimicrobials. At present, the
83
most efficacious treatment regimen, if any, is a fever or respiratory illness.

unknown. Suspected Case:
Limiting the spread of SARS: Respiratory illness of unknown etiology
Infection control precautions should be with onset since February 1, 2003, and the
continued for SARS patients for 10 days after following criteria:
respiratory symptoms and fever are gone. SARS Measured temperature > 100.5F (>38 C)
patients should limit interactions outside the AND
home and should not go to work, school, out-of-
One or more clinical findings of respiratory
home day care, or other public areas during the
illness (e.g. cough, shortness of breath,
10-day period.
difficulty breathing, hypoxia, or radiographic
During this 10-day period, all members of findings of either pneumonia or acute
the household with a SARS patient should respiratory distress syndrome) AND
carefully follow recommendations for hand
Travel within 10 days of onset of symptoms
hygiene, such as frequent hand washing or the
to an area with documented or suspected
use of alcohol-based hand rubs
community transmission of SARS (see list
Each patient with SARS should cover his below; excludes areas with secondary cases
or her mouth and nose with a tissue before limited to healthcare workers or direct
sneezing or coughing. If possible, a person household contacts)
recovering from SARS should wear a surgical
OR
mask during close contact with uninfected
persons. If the patient is unable to wear a surgical Close contact* within 10 days of onset of
mask, other people in the home should wear one symptoms with either a person with a
when in close contact with the patient. respiratory illness who traveled to a SARS
area or a person known to be a suspect SARS
Disposable gloves should be considered for
case.
any contact with body fluids from a SARS
patient. However, immediately after activities Close contact is defined as having cared for,
involving contact with body fluids, gloves should having lived with, or having direct contact
be removed and discarded, and hands should be with respiratory secretions and/or body
washed. Gloves should not be washed or reused, fluids of a patient known to be suspect SARS
and are not intended to replace proper hand case.
hygiene Areas with documented or suspected
SARS patients should avoid sharing eating community transmission of SARS: Peoples
utensils, towels, and bedding with other members Republic of China (i.e., mainland China and
of the household, although these items can be Hong Kong Special Administrative Region);
used by others after routine cleaning, such as Hanoi, Vietnam; and Singapore
washing or laundering with soap and hot water. Note: Suspect cases with either radiographic
Common household cleaners are sufficient evidence of pneumonia or respiratory
for disinfecting toilets, sinks, and other surfaces distress syndrome; or evidence of
touched by patients with SARS, but the cleaners unexplained respiratory distress syndrome
must be used frequently.Other members of the by autopsy are designated probable cases
household need not restrict their outside activities by the WHO case definition.
unless they develop symptoms of SARS, such as Source : Centre for disease control, Atlanta, USA.
84
2003; 5(2):173
PRACTITIONERS COLUMN
TIPS FOR PRACTISING Before the arrival of a patient

PEDIATRICIAN Our goal is to cure sometimes, relieve often
but comfort always
*Kamlesh R. Lala
**Mrudula K. Lala Do not prescribe without examining the
patient. Discourage telephonic advice.
Today doctors are practising under the Tackle diplomatically.
constant pressure of the society which now a On arrival of a patient
days see the doctor not as a GOD or messiah, but
Receive the patient with a smile.
simply a professional and has expectations
beyond our abilities to fulfill. Practising doctor See the patient as one of your relatives.
always try to do away with professional hazards Look at him as a Patient only without
of allegation of unlawful activity and its taking into consideration caste, religion,
sequelae. Following are the tips for the practicing race, politics etc.
pediatrisian. Address the patient by his first name which
he likes the most.
Personality of a doctor
Letterhead
Doctor is expected to be gentle, soft spoken,
Mention your qualifications and designation
well behaved and noble.
on the prescription.
Do not smoke or chew pan masala in front Qualification means recognized degree/
of patient diploma as regulated by Indian Medical
Dont be overconfident. Never challenge Degree Act 1916, as amended from time to
your colleague. Never criticize. time.
Do not examine the patient when you are Mentioning of scholarship, membership and
sick, exhausted or under the influence of awards should be avoided.
drug or alcohol. Mention complete address, your registration
number and telephone numbers along with
Develop empathy towards patients. Dont
timings.
get emotionally attached.
Registration of the patient
Never talk loose of your colleagues despite
intense professional rivalry. Always mention date and time of
consultation. Mention the relation of
Never try to challenge anybody. informant to patient.
Mention age, sex, weight of the patient.
* Consulting Pediatrician
Enter address and contact number of the
** Assistant Professor
Department of preventive and social medicine
patient
B. J. Medical College, Ahmedabad If referred, look at the referring note.
85
History taking Doctor patient relationship

Look carefully. Listen to the patient Try to establish healthy doctor-patient
attentively. Ask questions intelligently. relationship.
Ensure privacy and confidentiality even Professional courtesy, confidence and
during history taking. courage to tell the truth, coordination,
Always face the patient. Maintain eye communication, creative and imaginative
contact that is comfortable to the patient. Do thoughts; are all the bridges between doctor
not stare. and patient.
Check for pregnancy, lactation or any other Problem arises only when there is lack of a
pediatric chronic disease. proper interaction between doctor, patient
and relatives.
Take history of drugs being taken. Record
history of drug allergy. Try to understand the feelings of patients and
try to convince them that you are doing /
Take family history.
trying the best.
If the patient or relatives are erring on any
account like history not giving a reliable, Do not talk to any angry patient or relative
refusing investigations, refusing admission, about any other subject until you understand
make a note of it or seek written refusal the reason for the same. Take necessary time
preferably in local language. and steps to calm him down. Be patient with
patients. Patients are always impatient.
Examination
Investigations
Ensure privacy.
Investigation is not a substitute for your
Always ensure the presence of a relative or
clinical judgement. They are only
female attendant before examining a female
supportive.
patient, even if she is your regular patient.
Never neglect patients complaints. Refer to standard diagnostic centre or to a
qualified doctor.
Expose completely the part to be examined
or you may miss something. Routinely advise X - rays in injury to bones
and joints.
Always put your hand on the part that the
patient says is painful, last. Consider the affordability of the patient. Do
Do a complete examination including only those investigations which are
ausculation. indicated.
If after completing the examination, the Remember to advise in writing the
patient or attendant feels that something has investigations required for periodic
been left out or wants something to be evaluation e.g. blood sugar estimation in
reexamined, oblige him. diabetes mellitus, CBC in anti cancer drugs,
LFT in hepatotoxic drugs and so on.
Ask the patient to come back for review the
next day, in case you have examined him Always read reports, interpret the results and
hurriedly or if you are not sure. make a note of it.
86
2003; 5(2):175
Conclusion Consent
Never label any condition functional unless Always obtain a legally valid and informed
exhaustive examination and investigations consent before any surgical or invasive
rule out any organic cause. diagnostic procedure.
Do not give high hopes, do not guarantee Denial of consent should always be
cure and be careful while informing the mentioned.
patient or the relatives about prognosis.
Consent does not give blanket immunity to
Let the patient know the diagnosis and
a doctor. It is not a protection against
treatment of the condition he is suffering
negligence.
from.
Records Treatment
Records should be genuine and not Do not do anything beyond your level of
manipulated. competence i.e. qualification, training and
experience.
Records are a must for medico legal, income
tax and consumer protection purposes. A Always check and recheck injection and
well kept record is your best friend and best vaccines for name and expiry date. Also
defence in the court. reconfirm the route of administration.
Brief, incomplete and cryptic records are of Always use disposables or confirm
no use in courts. sterilization.
Records should not be exhaustive, but should Avoid unnecessary injections, IV drugs and
be brief yet informative and complementary drips. If I will not do it, somebody else
to management. would do it is a wrong way to think.
Records are based on facts and not on Justify indication of treatment and
memory and expected findings. Maintain the procedures.
record and not simply keep them.
In case of any deviation from standard care,
Medical records for inpatients are to be kept
mention reasons.
and maintained for three years from the date
of commencement of treatment. In an agitated child, restrain him properly
Keep complete medical record of history, with assistants to avoid needle being broken
clinical findings, diagnosis, investigations inside.
and treatment etc. In case a particular drug or equipment is
In complicated cases record precisely the not available, make a note.
history of illness and substantiate physical Try to avoid even a minor procedure under
findings on your prescription. local anesthesia in a consulting room.
Mention Diagnosis under review or under Inpatient
investigation until diagnosis is finally
settled. An inpatients file should contain
Non willingness of the patient for Case papers right from the day of admission
investigation and admission should be with every personal detail. All examinations
mentioned in local language. carried out and positive findings.
87
If there are no positive findings, mention No Remember:

complaints. GC good. Explain the prognosis and mention it.
Investigations carried out and their reports. Give alternate contact number in case of
Date and time of daily check up and serial emergency and non availability.
follow up in critical patient.
Dont forget to provide genetic counselling
Record daily vital parameters. to couples and parents with known family
Prescription history of children having genetic
abnormalities.
General :
Referring the patient
Avoid writing ayurvedic formulations.
Create, build and strengthen your
Do not allow substitute from chemist. relationship with practitioners of your area
Remember major drug interactions and so that they may be of help in crisis.
special situations like pregnancy and Prepare a list of specialists and super
lactation. specialists whom you trust and have a good
Write names of drugs clearly using capitals, rapport.
to avoid confusion with similarly spelled
Always provide a referring note and if
drugs.
possible make a phone call. This is liked by
Write in a sequential manner e.g. main drug the patient and shows your concern for him.
first, then supportive and lastly vitamins.
Keep a record of this reference.
Use correct dose. Do not over prescribe (too
If you are not sure about the diagnosis, do
many drugs, larger dose, for longer duration)
not hesitate to get the second opinion or to
or under prescribe.
discuss the case with a friend
Mention mode, interval of administration
Patients have the right for the second
and instructions in local language.
opinion. Let the patient choose another
Specifically mention review and follow up doctor if he wishes so.
schedule.
Discharge
Instructions:
Never refuse discharge against medical
Explain likely side effects of drug and the advise, as it is his right. Mention it and take
actions to be taken if they occur. his signature.
In chronic ailments, mention treatment to be Issue a discharge card with every detail of
taken immediately in case of emergency e.g. illness, investigation, treatment, procedures
diazepam in seizures, paracetamol in fever, done etc. for future reference.
antispasmodics in renal colics and so on.
Also mention treatment to be taken, follow
Mention additional precautions e.g. food, up schedule, advice for diet and exercise.
rest, avoidance of certain drugs etc. if
indicated. When asked for a written document, never
hesitate to give them. But be careful to enter
Advise accordingly if dose is to be tapered all appropriate details before handing over
before stopping. them.
88
2003; 5(2):177
Death of a patient Sickness certificate:

Do not leave at the moment of death. Your 1. Never give back dated certificates.
presence and experience are most needed.
Remember that the relatives have sustained 2. Never give it without seeing the patient.
a grievous loss. 3. Fitness certificate should be issued by a
Keep cool, ignore the comments of others, doctor who has issued illness certificate.
and call for assistance if needed.
4. Never forecast fitness in advance.
It would and should appear human if you
forego the fees for the incident that has Death certificate:
triggered off the situation. 1. Do not issue it unless you yourself have
Payments verified it and the patient was under your
care for recent illness.
When patient comes, doctor is GOD
When doctor treats him, it is his DUTY. 2. State clearly the cause of death and if in
doubt ask for police help.
When bill comes, doctor is a DEMON
3. It is to be issued free of charge.
So be careful while giving a bill or if possible
tell him rough estimate before starting Fees Receipts:
treatment or giving costly vaccines.
1. Never issue false receipts of fees received.
According to new rules by MCI, a doctor
should display his fees and other charges. 2. Should be printed, in duplicate and serially
numbered as required by income tax
Never be rigid for charges as most of the
department.
troubles start with this only. A craze for
money should not be there. 3. Take the signature of the patient on the
Do not refuse the patients right to examine duplicate.
and receive an explanation about your bill. CME
Certificates Remember Eyes cannot see what mind does
General: not know.
1. Issuing certificate is a tricky job. Regularly attend CME to update your
2. Use proforma given by MCI. Write your knowledge and skill. There is no limit to
registration number clearly. knowledge. So even if you feel, you know,
you learn and you will find something new
3. Keep duplicate record. Instead of keeping
every time.
carbon copy on a plain paper, keep both the
pages same. Keep with you and refer at times, the latest
4. Always take signature or left thumb edition of the standard textbook of your
impression on certificate. branch.
5. You can charge reasonable amount for the Always attend at least two updates and
certificate. conferences every year.
6. Avoid false certificates diplomatically. Subscribe at least to one update / journal.
89
Set up Remember that practice of truth is not only

good as a principle but it is good as a policy.
Should have efficient, competent and fast
working manpower. When you have the slightest doubt of
litigation, complete all data meticulously and
Update knowledge and skill of your staff
preserve them.
also.
Never fail to seek proper legal and medical
Update facilities and equipments according
advice before filing reply to the notice
to prevailing current standards in your area.
received from consumer court.
Do not purchase costly sophisticated
Never entertain compromise as it may lead
equipments only for the sake of prestige. It
to blackmailing in future and a life time
may induce you to indulge in malpractice.
tension.
Time management
Never give away any original to any
Efforts should be there to have fixed authorities. You may give a xerox copy.
consulting hours. Those having an overflow
This is not an exhaustive account, but is
of outpatients, should keep appointment
meant for general information. It is needless to
system.
mention that each situation needs its own
Discourage home visits as far as possible, consideration. One will face different situation
except in emergency. every time and so there cannot be the rule of
Give fixed time slot for representatives thumb.
during your consultation hours. Bibliography
Never encourage relatives and visitors
1. Vaidya Jatin P. Doctor in law. Gujarat Med J
during your busy consulting hours.
1988; 34(1): 21-25.
Do not forget the importance of your 2. Shah K K and Mehta H P. Doctor and law. pub-
physical fitness. Allot at least 20-30 minutes lished by IMA Gujarat State branch, 1994.
for yourself and your family in your
3. Jagdish Singh. Dos and donts for Pediatri-
appointment diary.
cians, IAP J practical pediatr, 1996; 4(3):
Take a weekly off to meet social 203-207.
commitments and refreshment with your 4. Jagdish Deshpande. Healthy tips for medical
family. practice, Knoll pharmaceuticals publication,
Take a vacation once or twice a year. 2002; pp 1-3.
5. Ajay Agrawal. Time management in General
Litigation
Practice, Family medicine India (IMACGP).
When healthy doctor-patient relationship can 1996; 1(1):31-32.
be created, evolved, nurtured and 6. Family Medicine India (IMACGP), Medical
strengthened, no doctor has to fear about any records for Doctors A must 2001; 5(2): 18-
litigation. 22.
Irritable nature, arrogance and high handed 7. Insight, The consumer magazine, September
approach by doctor are his enemies. 2002; 22(5): 1.
90
2003; 5(2):179
QUESTIONS AND ANSWERS
Q.No. 1: Male / 7Yrs. H/O Puffiness and followed by appropriate management depending
oedema abdominal wall 7 days. Oliguria. B.P on the histology would be the ideal approach.
120/92. No headache/vomiting.Vitals Stable. Alternatively a course of steroids may be given
Lab: Urine Alb +++ three/four days if parents are not willing for kidney biopsy even
consecutive. 8-10 RBC. Granular cast. Blood after adequate explanation about the need of it
Urea-104. S.Creatinine 1.4. S.Cholesterol 360. for diagnosis and management.
C3-Normal. Xray Chest Normal. USG
Abd.Normal. Dr. M.Vijayakumar,
Consultant Pediatric Nephrologist,
What are the D/D?
? MCNS (High Cholesterol) Chennai 600 034.
? MPGN Presenting as NS.
? Nephrotic range of protienuria with Acute Q. No.2 i) Rabies can be caused by bite of all
Glomerulonephritis. (High Creatinine High animals except Rat. Is it true?
B.P.RBC and granular cast in urine.)
ii) Rabies rare to be transmitted by Rat.
Please guide. Shall we
Harrisons TB of internal medicine.
1. Wait As now S.Creatinine has gone down
to Dr.Sanjeev Aggarwal
0.9.(Patient is on Amoxy+Lasix+Nifedipine) Chandigarh.
2. Kidney Biopsy?
A. No.2. In the Red Book 2000 edition of the
3. Start Steroid considering NS.
American Academy of Pediatrics Committee on
Dr. H.K.Takvani, Infectious Diseases under the heading zoonoses
Children Hospital and Neonatal Care Centre, ( Diseases transmitted by Animals) page no.776,
Jamnagar, Gujarat. Appendix vii Common animal sources for Rabies
transmission are stated as Dogs, Cats, Ferrets,
A. No.1. This child is presenting with combined Bats, Skunks, Foxes & Wood Chucks and the
features of nephritic syndrome and nephrotic mode of transmission is by bites. Hence Rat
syndrome. Serum total protein and serum Bites do not transmit rabies.
albumin values are not available. Lower levels
will support the diagnosis of nephrotic syndrome. Dr.A.Parthasarathy,
A combination of nephritic and nephrotic Retd. Senior Clinical Professor of Pediatrics,
syndrome, hypertension, renal failure and age of Madras Medical College,
more than 5 years indicate a very high possibility Deputy Superintendent, Institute of Child
of non-minimal change disease. Renal biopsy Health & Hospital for Children.Chennai.
91
Q. No.3. Indrawing in respiratory distress. In obstruction or poorly complaint lungs.

case of respiratory distress, suprasternal, Suprasternal hollowing are especially striking in
intercostal and subcostal indrawing is seen. Is extrathoracic airway obstruction, in which the
it a real indrawing or a visual deception? My large negative intrathoracic pressure that
perception is that because of the underlying attempts to overcome the obstruction, results in
pathology, lung expansion is restricted, while collapse of extrathoracic airways. Intracostal
the thoracic cage moves out normally causing retraction is a sign of increased lung stiffness or
the illusion that some portion of the thoracic increased work of breathing due to airway
cage is moving inwards. Why does the obstruction. Subcostal retractions are always a
indrawing occur? sign of hyperinflation and a flattened diaphragm
due to small airway obstruction. Normally when
Dr.Yash Paul, the dome shaped diaphragm contracts and moves
Consultant Pediatrician, Jaipur 302 016. into the abdomen, the lower edge of the ribcage
to which the anterior edge of the diaphragm is
A. No.3. Normal breathing is effortless. attached, moves upward and outward. In the
Breathlessness, dyspnoea, respiratory distress, all presence of hyperinflation, the diaphragm is
means increased work of breathing, characterized depressed and when it contracts and moves
by sub-costal retractions, intercostal retractions, further into the abdomen, it pulls the lower edge
suprasternal hollowing and flaring of alae nasi. of the ribcage inwards resulting in subcostal
Flaring of alae nasi is a sign of increased airway retractions. Clinically, the presence of subcostal
resistance with breathing through the nose. 50% retraction means hyperinflation, when retraction
of the resistance of airflow occurs in the nose is worsening, small airway obstruction is
whereas the other 50% occurs in the large worsening; when retraction is decreasing in
airways. With obstruction in the airways the severity, the degree of air trapping is lessening.
infant can decrease total airway resistance by So indrawing in respiratory distress is a real
flaring the alae nasi and thus decreasing the indrawing and not a visual deception.
resistance in the nose. Retractions is a sign of
increased work of breathing. Normally tidal Dr.L.Subramanyam,
volume breathing generates a negative Consultant in Pediatric Pulmonology,
intrathoracic pressure of 4-5 cms of water. Kanchi Kamakoti CHILDS Trust Hospital,
Retractions occur when the negative intrathoracic Chennai 600 034.
pressure is increased as in individuals with airway
CONTRIBUTORS TO CORPUS FUND OF IJPP
Rs. 1000/- Rs. 500/-

Dr. V.P.Anitha, Chennai, Tamilnadu
Dr. B.I. Sasireka, Chennai, Tamilnadu
Dr. L.S. Kadam, Pune, Maharashtra
Dr. Suresh K. Kakhandaki, Bagalkot, Karnataka Dr. Mahesh Patel, Surat, Gujarat
Dr. K.K. Agarwal, Rajasthan Dr. S. Bose, Durgapur, West Bengal
Dr. H.V. Kotturesha, Shimoga, Karnataka
Dr. K.M. Manoharan, Chennai, Tamilnadu
Dr. K.H. Vimala, Bangalore, Karnataka
92
2003; 5(2):181
NEWS AND NOTES
The Rheumatology Chapter of IAP & Mumbai Branch Of IAP invite you to the
The 1st National Conference in Pediatric Rheumatology
ARTICULATIONS IN PEDIATRIC RHEUMATOLOGY
Venue: R D Choksi Auditorium,Golden Jubilee Building,Tata Memorial
Hospital,Parel,Mumbai 400013
Dates: Sat. 8th November-10am-6pm and Sun. 9th November-9am-4pm
Registrations-(last date 30.9.03limited to 200 only)No spot registrations
(includes delegate kit, course material, lunches and coffee breaks)
before 31.07.03 after 31.07.03
IAP Rheumatology Chapter Members Rs 800 Rs 1200
Others * Rs 1000 Rs 1500
Post graduates/Residents(40 only) Rs 600 Rs 900
(letter from HOD required)
Cheques/DD to be drawn favouring IAP Rheumatology Chapter payable in Mumbai (outstation
cheques add Rs. 50)
*enroll as Rheumatology Chapter Life Member(Life membership Rs500) and avail of cheaper
registration. Send separate cheque/DD. Refund last date 30.09 .03 -50% after conference. All payments
and correspondence to:
Dr Raju Khubchandani, 31 Kailas Darshan, Kennedy Bridge Mumbai 400007,
Tele O- (022) 23865522, R-(022)22028388, Fax(022) 23898362, Email [email protected]
cut here or attach a letter stating below facts
THE 1ST NATIONAL CONFERENCE IN PEDIATRIC RHEUMATOLOGY
ARTICULATIONS IN PAEDIATRIC RHEUMATOLOGY
PERSONAL DETAILS
Name:
Institution Designation
Consultant\Student Paediatrics\Other (specify)
Address (include pin code)
Phone: Res( ) Mobile Fax:( ) Email
Food preference Veg\Non-veg
Need assistance with staying arrangements? Yes/No -We will mail you options
(e mail id essential)
Remittance Cheque/DD no Bank Branch
Signature
93
NEWS AND NOTES
SOUTH PEDICON 2003

XVII SOUTH ZONE CONFERENCE OF IAP
&
XXVIII ANNUAL CONFERENCE OF IAP - TNSC
Organized by
IAP North Arcot Branch &
Christian Medical College & Hospital, Vellore, Tamilnadu
Date : September 4,5 & 6, 2003.
Venue : Christian Medical College & Hospital, Vellore, Tamilnadu
Registration Fees
Category of Before Before Before From 1.8.2003 &
delegates 15.4.2003 31.5.2003 31.7.2003 Spot
IAP Member Rs.1000/- Rs. 1500/- Rs. 2000/- Rs.3000/-
Non IAP Member Rs. 1500/- Rs. 2000/- Rs. 2500/- Rs. 3250/-
PG student Rs. 900/- Rs. 1150/- Rs. 1400/- Rs. 1750/-
Accompanying person Rs. 1000/- Rs. 1500/- Rs.2000/- Rs. 3000/-
Foreign Delegates US$ 200 US$300 US$400 US$500
For preconference concurrent workshops (on 3rd September 2003) Rs.500/- each*
1. Intensive Care 2. Clinical Epidemiology 3. NALS
* Can register for only one workshop
For further details contact:

Dr. Chellam Kirubakaran,
Organising Secretary, South Pedicon 2003, Department of Pediatrics,
Christian Medical College & Hospital, Vellore, Tamilnadu 632 004.
Phone No. (0416) 2262603, 2221346
94
2003; 5(2):183
PEDICON 2004
41st NATIONAL CONFERENCE OF THE INDIAN ACADEMY OF PEDIATRICS
Healthy child - Mighty IIndia
ndia
8-11 January 2004, Chennai
Venue: Sri Ramachandra Medical College & Deemed University,
Porur, Chennai, Tamilnadu 600 116
The metropolitan city of Chennai known for its excellent hospitality and good ambience, invites
you to the 41st National Conference of the Indian Academy of Pediatrics at Sri Ramachandra
Medical College and Deemed University, Porur, Chennai between 8-11 January 2004. The theme
of the conference is Healthy child - Mighty India. The pediatricians of the city of Chennai
eagerly await to host this prestigious event after a span of 17 years by providing academic feast to
fellow pediatricians from India and abroad.
Dr. C.S. Rex Sargunam Dr. A. Balachandran Dr. M.P. Jeyapaul
Organising Chairman Organising Secretary Hon. Treasurer
Category Before Before Before Before From 1.12.2003

30.4.2003 30.08.2003 30.10.2003 30.11.2003 & Spot
IAP Member Rs.2300 Rs.2800 Rs.3500 Rs.4500 Rs.5500
Non IAP member Rs.2500 Rs.3000 Rs.3800 Rs.4800 Rs.6000
Accomp. person Rs.2000 Rs.2500 Rs.3000 Rs.4000 Rs.4500
PG Student # Rs.2000 Rs.2500 Rs.3000 Rs.4000 Rs.4500
Senior citizen ## Rs.2000 Rs.2500 Rs.3000 Rs.4000 Rs.4500
SAARC delegate Rs.2500 Rs.3000 Rs.3800 Rs.4800 Rs.6000
Foreign delegate US $ 200 US $ 250 US $ 350 US $ 400 US $ 500
Cancel/Refund * 70% 60% 50% 30% Nil
# PG student should submit the bonafide certificate

## Senior citizen > 65 years
* Refund will be done one month after the conference
** Details regarding the preconference workshops (on 7.1.2004) will be intimated later
For further details contact:
Dr. A. Balachandran,
Organising Secretary, PEDICON 2004,
IAP - TNSC Flat, Ground Floor, F Blook, Halls Towers,
56 (Old No.33) Halls Raod, Egmore, Chennai 600 008.
Phone: 044-28190032, 28191524 Email: [email protected]
95
REGISTRATIONFORM
PEDICON2004
41st NATIONAL CONFERENCE OF INDIAN ACADEMY OF PEDIATRICS
Healthy child - Mighty IIndia
ndia
Venue: Sri Ramachandra Medical College & Deemed University, Porur, Chennai 600 116
Delegates Title [ ] Dr. [ ] Prof. [ ] Mr. [ ] Mrs. [ ] Ms. IAP Membership No...............................
Name ......................................................................................................................................................
Mailing address .....................................................................................................................................
Building/ Institution Name ....................................................................................................................
Flat/Room No. .......................................................................................................................................
Street Name ...........................................................................................................................................
Locality / Suburb ...................................................................................................................................
City / District .........................................................................................................................................
State .................................................................... Pin-code ...............................................................
Residence STD Code ......................................... Phone no. ..............................................................
Clinic/Hospital STD Code ................................. Phone no. ..............................................................
Fax No. ............................. Mobile No. ................................. Pager No. ..............................................
E-mail ....................................................................................................................................................
Food Preference [ ] Vegetarian [ ] Non vegetarian CME : Yes / No
IAP Delegate Fees Rs. .....................................................
Non-IAP Delegate Fees Rs. .....................................................
PG Student Fees Rs. .....................................................
Accompanying person Rs. .....................................................
Others Rs. .....................................................
Demand Draft Number............................................. of .......................................... Bank
.......................................... Branch dated ...............................
Please Note: Signature
* CME is free, however, prior registration is a must.
* Please send your payment only by demand draft. Please do not send any cash by post. Please do
not use cheque for payment. Demand draft to be made in favour of Pedicon 2004, payable at
Chennai.
* Please mail the registration form along with the Demand Draft by registered post or by courier only
to the following address.
Secretariat:
Dr. A.Balachandran, Organizing Secretary, PEDICON 2004,
IAP-TNSC Flat, Ground Floor, F Block, Halls Towers, 56 (Old No.33) Halls Road, Egmore,
Chennai-600 008. Tamilnadu, India.
Phone: 28190032, 28191524, Email: [email protected]
96
2003; 5(2):185
INDIAN JOURNAL OF PRACTICAL PEDIATRICS

ADVERTISEMENT TARIFF
Official Journal of the Indian Academy of Pediatric - A quarterly medical journal
committed to practical pediatric problems and management update
Name ...................................................... FULL PAGE
Address .................................................. B/W Colour*

............................................................... Ordinary 4,000 8,000
............................................................... Back cover - 15,000
...............................................................
Second cover - 12,000
City ........................................................
Third cover - 12,000
State ............................... Pin ...............
** Gate fold / Insert - 6,000
* Positives of the advertisements should be given by the company.

** For gate fold and inserts the printed material should be given by the company.
I / We wish to advertise in the Indian Journal of Practical Pediatrics.
Signature
Kinldy send your payment by crossed demand draft only drawn in favour of
Indian Journal of Practical Pediatrics and art work / matter to
MANAGING EDITOR
Indian Journal of Practical Pediatrics
IAP-TNSC Flat, Ground Floor,
F Block, Halls Towers,
56 (Old No.33), Halls Road,
Egmore, Chennai - 600 008. India
Phone : 2819 0032
Email : [email protected]
9796
98
2003; 5(2):187
Indian Journal of
Practical Pediatrics
Subscription Journal of the
IJPP
Indian Academy of Pediatrics
JOURNAL COMMITTEE
NATIONAL ADVISORY BOARD
Editor-in-Chief
Dr. A.Balachandran President, IAP
Executive Editor
Dr. H.P.S.Sachdev
Dr. D.Vijayasekaran
Managing Editor
Dr. K.Nedunchelian President Elect, IAP
Associate Editors
Dr.M.K.C.Nair
Dr. N.C.Gowrishankar
Dr. Malathi Sathiyasekaran
Dr. P.Ramachandran
Editor, Indian Pediatrics
Dr. C.V.Ravisekar
Dr. Panna Choudhury
Dr. S.Thangavelu
Executive Members
Dr. B.Anbumani Members
Dr. Janani Shankar
Dr. Ashok K.Rai
Dr. P.S.Muralidharan
Dr. K.Nagaraju Dr. C.M.Chhajer
Dr. T.L.Ratnakumari Dr. S.R.Keshava Murthy
Dr. T. Ravikumar
Dr. Krishan Chugh
Dr. S.Shanthi
Dr. So.Shivbalan Dr. B.R.Nammalwar
Dr. V.Sripathi Dr. A.Parthasarathy
Dr. Nitin K.Shah
Dr. M.Vijayakumar
(Ex-officio)
Dr. Vijay N.Yewale
Indian Academy
of Pediatrics
Kailash Darshan,
Kennedy Bridge,
IAP Team - 2003 Mumbai - 400 007.
President Members of the Executive Board

Dr. H.P.S. Sachdev Dr. C.M.Aboobacker (Kerala)
Dr. Apurba Kumar Ghosh (West Bengal)
Dr. Ashok Kumar Gupta ( )
President Elect Dr. Ashok K.Rai ( )
Dr. M.K.C.Nair Dr. Baldev S.Prajapati (Gujarat)
Dr C.P.Bansal (Madhya Pradesh)
Dr. C.M. Chhajer (Tripura)
Imm. Past President Dr. M.Dasaradha Rami Reddy (Andhra Pradesh)
Dr. Dilip K.Mukherjee Dr. K.W.Deoras (Chhattisgarh)
Dr. D.Gunasingh (Tamil Nadu)
Dr. S.R.Keshava Murthy (Karnataka)
Vice President
Dr. Krishan Chugh (Delhi)
Dr. C.P.Bansal Dr. Mahesh Kumar Goel ( )
Dr. Manoranjan Sahay (Jharkhand)
Dr. Niranjan Mohanty (Orissa)
Secretary General
Dr. P.S.Patil (Maharashtra)
Dr. Nitin K. Shah Dr. K.R.Ravindran (Tamil Nadu)
Dr. Ramesh K.Yelsangikar (Karnataka)
Treasurer Dr. Sailesh G.Gupta (Maharashtra)
Dr. Satish V.Pandya (Gujarat)
Dr. Bharat R. Agarwal Dr. (Mrs). Shabina Ahmed (Assam)
Dr.Shahshi B.P.Singh (Bihar)
Editor-in-Chief, IP Dr. S.C.Singhal (Haryana)
Dr. B.K.Sudhindra (Kerala)
Dr. Panna Choudhury
Dr. Sudesh K.Sharma (Punjab)
Dr. Suil Gomber (Delhi)
Editor-in-Chief, IJPP Dr. Tapan Kumar Ghosh (West Bengal)
Dr. Vijay N.Yewale (Maharashtra)
Dr. A. Balachandran
Dr. R.Virudhagiri (Tamil Nadu)
Dr. Yashwant Patil (Maharashtra)
Joint Secretary
Dr. D.Vijayasekaran
2003; 5(2):189
CONTRIBUTORS TO CORPUS FUND OF IJPP
Rs. 1000/- Rs. 500/-

Dr. V.P.Anitha, Chennai, Tamilnadu
Dr. B.I. Sasireka, Chennai, Tamilnadu
Dr. L.S. Kadam, Pune, Maharashtra
Dr. Suresh K. Kakhandaki, Bagalkot, Karnataka Dr. Mahesh Patel, Surat, Gujarat
Dr. K.K. Agarwal, Rajasthan Dr. S. Bose, Durgapur, West Bengal
Dr. H.V. Kotturesha, Shimoga, Karnataka
Dr. K.M. Manoharan, Chennai, Tamilnadu
Dr. K.H. Vimala, Bangalore, Karnataka

Vol.5 No.2

Uploaded by

Copyright:

Available Formats

Vol.5 No.2

Uploaded by

Document Information

Original Description:

Copyright

Available Formats

Share this document

Share or Embed Document

Sharing Options

Did you find this document useful?

Is this content inappropriate?

Copyright:

Available Formats

Vol.5 No.2

Uploaded by

Copyright:

Available Formats

2003; 5(2):89

INDIAN JOURNAL OF IJPP

Vol.5 No.2 APRIL-JUNE 2003

Laboratory evaluation of hypertension 133

NEWS & NOTES

1ST NATIONAL CME ON INFECTIOUS DISEASES IN CHILDREN:

GUIDELINES FOR where the pathogen is most likely to be found

Agents of pneumonia in an immuno- area again, without sterile gloves. Collect

oxygen. Therefore samples collected without Blood for Serology

NEWS AND NOTES

USE OF IAP LOGO

INTERPRETATION OF The interpretation of various investigations will

Table 1. Interpretation of ELISA for rubella specific antibodies

2. Cytomegalovirus Isolation of virus or demonstration of CMV

3. Herpes simplex virus Virus co-culture, p 24 antigen detection ,

Diagnosis repeat IgM concentration shows a fall in

NEWS AND NOTES

5TH NATIONAL CONGRESS ON PEDIATRIC CRITICAL CARE

DIAGNOSTIC APPROACH TO THE presenting with rheumatologic symptoms will

Traumatic injury of intracapsular structure:

Fig 1: Algorithm for approach to child with acute arthritis.

Systemic arthritis Polyarthritis (RF positive)

Other arthritis Table 3: Standard Anti-inflammatory

recurrent painless effusions which, on aspiration, References

RAPID DIAGNOSTIC TESTS An ideal diagnostic tool needs to fulfill certain

* Saranya N It should be easy to perform

Table 1. Properties of diagnostic tests used in typhoid fever

Table 2. Properties of tests used in diagnosis of malaria

tests are useful for screening and confirmation Dengue

In summary, there is no single perfect b) Molecular techniques like PCR, RAPD,

suspicion and a positive AFB smear will be to prevent vertical transmission

NEONATAL METABOLIC with untreatable disorders, it is important to

Acute neonatal encephalopathy

Non-metabolic causes Metabolic cause

Septicemia 1. Do Blood Ammonia

Severe metabolic acidosis Normal/Resp. alkalosis

3.Do Lactate, Pyruvate, Ketones

Mixed Keto-Lactic Acidosis Lactic acidosis only

Pyruvate dehydrogenase deficiency 5.Beta(OH)Butyrate/AcetoAcetate ratio

< 2:1 > 2:1

Pyruvate Carboxylase def Resp Chain Defects

i) CBC for neutropenia and thrombocytopenia lactic acidosis

Table 2. Laboratory tests

Screening Laboratory Tests Confirmatory Tests

Table 3. Differential diagnosis of common metabolic disorders:

gm/kg/day) to prevent endogenous protein References

LABORATORY DIAGNOSIS OF Lab diagnosis of persistent diarrhea

Treat dehydration and any associated systemic infection

A skilled microscopist and careful attention

Table 1. Causes of chronic diarrhea in different age groups

upto 12% of immunocompetent children with Kinyoun acid fast stain

Fig 2. Cryptosporidium oocysts seen in Fig 3. Characteristic fluorescent honey-

NEWS AND NOTES

LABORATORY EVALUATION OF secondary unless proved otherwise and every step

Definition of hypertension4 Table 1. Common causes of childhood

Severe proteinuria and hematuria suggests On documentation of sustained hypertension

renovascular disease is difficult to diagnose with Selective renal angiography

CT angiography hyporeninemia along with hyperkalemic

induced/related hypertension is the diagnosis essentially renal parenchymal hypertension.