mm

Hyperventilation provokes absence seizures

3 Hz spike and waves
DOC : ethoxsuccimidev

FPl-F7
Jt7-T:J
T S-TS
T5-0t
Fl"l-1'3
FS-Cl
C3-Pl
1'3-01
FP2-F-t
,·.a-c.-
c ....l'4
P+-02
FP:t--FI --.,......,~.......
.,._T4
T<l-T6
- -
T602 •v-~

J UVENILE MYOCLONIC EPILEPSY

Poly spike and wave discharges

MC generalised epilepsy in adolescents and young adults
DOC - leviteracetam f /b valproate

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 mm)

DOC generalised epilepsy: leviteracetam

DOC for focal epilepsy - oxcarbazepine > carbazepine

NEPHROLOGY
UTI AND RECURRENT UTI
st
Protocol for imaging : 1 episode of
8i1ateril reNI qnesisor UTI
bliteril rrdticystic 1 Pottlrfacles: - < lyr: USG, DMSA, MCU
dysplastic~ l.oH(m - 1-5yr: USG+ DMSA, if any
Bealnost abnormal MCU
Prunilentepimhic - >5yr: USG, if abnormal then
ro.tswldownwird
DMSA
P.eckJced fetal urine slintto eyes
- Recurrent UTI do all
excretion
- MCC- VUR, diagnosed by
Pmnonaryhypoplasia MCU
ausing lespRIO!y
bire

Linh debmilifs

POTTERS SYNDROME

I,
I I

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ISPN NEPHROTIC SYNDROME GUIDELINES 2021

1st episode : prednisolone for 12 wks
Frequent relapse: low dose, alternate prednisolone, if doesnt respondoral levamisole
Steroid dependent: low dose alternate prednisolone, f /b mycofenolate mofet il, if toxic
t hen cyclophosphamide for 12wks
Steroid resist ant: no r esponse to prednisolone for 6wks, DOC : tacrolimus f lb
cyclosporine

HYPOCHLOREMIC , HYPOKALEMIC METABOLI ACIDOSIS

POLYURIA N ormal urine output Hypertension
Barter syndrome Cystic fibrosis Liddle syndrome : ENaC
Gitelmann syndrome Pyloric stenosis
Vomiting
Diuretics

'l.I
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 PEDIATRICS 2
RECURRENT PNEUMONIA

Involvement : Same Sin le Lobe Multi1ile/Different Lobes

Anatomical malformations or Foreign body Genetic causes - Cystic fibrosis
Lobar emphysema Sickle cell anemia
Pulmonary sequestration Disorders of Cilia
Deficient immunity - HIV
Gastroesophageal reflux
Prim ry immunodeficiency.

CEREBRAL PALSY

Periventricular Kernicterus Term Asphyxia

Encephalomalacia: Leucomalacia: Affecting: Involving
Hemiplegic Spastic Diplegic Globus Pallidus. Thalamus and
Cerebral Palsy Cerebral Palsy t>yskinetic Posterior
Cerepral ,Palsy Putamen: Dyskinetic
Cerebral Pal

ENCEPHALITIS

Herpes S implex Varice lla Zoster ' Japanese Encephalitis

Involves inferior and medial Encephalitis and acute Symmetric or asymmetric
Regions of the temporal lobes cerebellar Ataxia bilateral thalamic
and the orbital gyri of the Spares basal ganglia hypodensities
frontal lobes

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 M YOCLON US
-E-p-ile_p_t-ic_ _ _ _ _ _ ___

Dravet syndrome Opsoclonus Subacute

Lennox gastaut syndrome Myoclonus ataxia syndrome sclerosing
Rett syndrome (paraneoplastic syndrome panencephalitis
Juvenile myoclonic epilepsy seen in neuroblastoma)
Dancing leg syndrome

POST INFECTIOUS NEUROLOGICAL SEQUELAE

Subacute sclerosing Sydenham's chorea Post tubercular meningitis

panencephal itis
Occurs 7-10 Years after Chorea Hydrocephalus
Wild type measles Hypotonia Basal exudates
infection Emotional changes Infarction ,
Presence of myoclonus in
stage f

FOET A L AND NEONATAL HYDROCEPHALUS / ANTEN ATAL HYDROCEPH A LUS

Malformations Intrauterine Infection

1 . Aqueductal stenosis 1. Toxoplasma
2 . Arnold Chiari II malformation 2. Cytomegalovirus
3.

H YDROCEPH A LUS IN OLDE R CHILD REN

Posterior fossa tumors ]nf ections

Medulfoblastoma ----------'"'""-----"-" 1. Cysticercosis
Pilocytic astrocytoma 2. Tuberculosis
E~end moma

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14 year old with no prior medical history old boy with complains of headache for t he past 6
months. On physical examination there is no abnormal f inding. On CT scan t here is enlargement
of lateral ventricles and third ventricle. On CSF examination there is normal opening pressure,
normal glucose and no leukocytes. Out of the following most likely cause is:

a) Aqueductal stenosis
b) Tubercular meningitis ·
c) Ependymoma
d) Cerebralabscess
Normal glucose r/o infections
~ ~ ~~ ~ -~ -~ - -- -- - - - - -- - -- - -- -
Duchenne Muscular Classical Myotonic
Q~ht _ _ ___ _ Qy_st_r-0JJ!tyjtyp_1:_!)______ _
X linked recessive • Autosomal dominant'-
Involves proximal (CTG repeats)
muscle • Involves distal
Dilated muscle
cardiomyopathy • Facial and
No involvement of Ext raocular muscle
Facial and affected
extraocular muscle • Ca~diac arrhythmias

D RUGS USED IN NEUROCUT A ENOUS S YNDRO M E

Selumetinib Symptomatic inoperable plexiform NF - 1

neurofibroma
Everolimus
--------,
Subependymal Giant Tuberous sclerosis
cell Astrocytoma
Renal ang iomy_2.
__li,_,
po_m_a____ __ ____,] Tuberous scler osis
'''
. Pulmonary lymphangioleiomyomatosis Tuberous scleros is
r--------1

I~

i~
SEGA Plexiform Neurof ibrorna

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 BRAIN TUMOR
CONGENITAL

Are diagnosed antenatally or within the

first 2 months of life
Most common type Teratomci (germ cell
tumour)
Choroid plexus papillomas which can cause
I
hydrocephalus

CHILDREN
M·ost, common
I
is .pifocyt
.
ic·a~·t,~~cy-toma (WHO , Medulloblastomci - MC-malignant
'grade,1) . ' , · o Embryonal tumo~.s ···
- Main! arises from The cerebellum o Arises from cerebellum ,

Ependymoma: Arises from the floor of 4th ventricle, therefore can cause obstruction 1

8 year old boy has histol')'. of headache with blurred vision for last 6 months. On examination
height is below 3%. On ophthalmological examination there is ,bilateral optic nerve atrophy.
Out of the foll~wing most likely diagnosis is:

a. Meningioma
b. Pituitary gland hyperplasia
c. Pituitary adenoma
d. Craniopharyngioma

Mass in the pituitary gland + visual

':defects
I
I
I I
'
\ ,,·
I

I
Hypqrunctio.n Hyperfunction
Crani9phoryngi9ma. Pituitary adenoma
I >

I I >

Since below 3% - not adenoma or hyperplasia

' > I

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 Delayedruberty
Setvm S..,

I
lowr and F5H HW.,Ufaacf'5t«
+
..!. .
~·
Gnllff ~Lado,-i test
'~ .

Norri,
+ .
furn«'~

~fnpwth +
MIII Bnlin
'Syndrome fMU~
0, Prim1rt tfttio•lu
llidJllilllft~

I ~,~,
llriefefter", f.ailure
Or·
PtitMry etv.ariMt

CJIS Twnour
i
Abs.Mao#
i
Normal
f.tilunt (acnal 1 1 ~ )

-~
cranloph~ olbctcwy ~k
Pituit.afy bulb/wlcn QUll!i
henlDtiderom +
kllmu,,

1st test: serum gonadotrophin level

POU1F1 or PIT1 gene - multiple pituitary hormone deficiency

PRECOCIOUS PUBERTY + CAFE-AU- LAIT MACULES

Visual field defects N o visual field defect

N eurof ibromatosis 1 McCun.e Albright syndrome
Due to optic nerve glioma GNAS rt\utation .
Central precocious puberty Peripheral precocious puberty

M UCOCU T AN!:OU S H YPERPIGMENT ATION +

I NTESTIN AL POLYPS

Peutz J eghers syndrome

Sex cord tumour with annular tubules
Increased aromatase activity - increase
estrogen

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 \,

''
I

Increased
11
aromatase
activity '
'
I I

I I

-- I•

Male _ Female

I
I
I
I

I
. I

Peripheral Peripheral
Precocious Contrasexual Precocious Isosexual
Puberty Puberty

P RE COCIOUS P UBERTY ( B O NE AG E < C HRO N OLOGIC AL A GE )

Hypothyroidism

s ill over
CONG ENITA L H YPOTHY ROID I S M

,Scree~lng for Congenital Hypothyroidism

(AIIMS protocol of neonatology)
(using T4 and TSH)

i
TSH80,nU/l
i
TSH > ~ - II) mU/l
l
l'SH 20 - 40 mU/t
' I

+ De<JNSeTII

!
Immediately st.Jrt the
+OecrN MH

!
Repot T'SH t T4 and
l
Jlepeilt TSH "14 .- If ,xmibleof

ttt.itment If possa,le of fl'C rt 4 at 7'-10 d.Jys of Ufe

Aboounat
! i
~ > 20m0/l in<2~of

!
Start trN-tmcmt

St.in treatment

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COMMON SYNDROMES ASSOCIATED WITH CARDIAC DISEASE

Macr ogloss ia -
due to myxedma
of t ongue

Will iams Syndrome

Turner Syndrome Mouth pen with decr eased teeth
Bicuspid aortic voice > aort ic Supravalvu lar aortic stenosis ,
vave disease> COA > AS peripheral PS, renal ar tery stenosis
>HCHS

Downs syndrome Noonan syndrome Alalgile syndrome - pointed

ECD PS chin, pu lmonar-r- st enosis
pallor plus hematosplenomegaly
Prominence of maxillary bones - haemolytic facies
Thalassemia
Pale, green. Bronze, yellow - greedindh
discolouration

DRUGS I N HE M ATOLOGY

Thalassemia 1· Anti-apoptotic • Approved for patients >

• Luspatercept 12years

A
i li~
Hemophilia A · Emicizlmib FVllla
• Emicizumab

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DR UGS I N SI CKLE CELL ANE MI A

~ -- ~ - - - - -- -- -~ ~ - - - - - - - -
Crizanlizumab Voxelotor •
Anti P Selectin antibod Inhibitor of deoX enated sickle Hb ol merization

......
Dw;a t 1"1C

Clinical manifestation of Iron Deficiency Anemia

PICA
Pagophagia (craving for ice cubes)
Magpie bird with the Latin name Pica
Breath holding spell
· Febrile seizures

-
ILi•••-·
RE SP ONSE TO IRON TH ERAPY I N IRON DEF I CIENCY ANEMIA
~- Paper

12 - 24 hours Increased appetite and decreased irritability due to replacement of

intracellular iron enzymes
36-48 hours Initial bone marrow res onse
48 - 72hours Reticuloc tosis (1 st res onse in eri heral blood)
4-30 days Increase in hemoglobin level
Increase in f erritin

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lgM - Low IgA-decreased
IgG-Normal IgG2-decreased
IgA-elevated IgG4-decreased
IgE-elevated (allergic) lgE - decreased
Thrombocytopenia present Increased incidence of
o Hodgkin disease
o Leukemia
o L m homo

NON-INFECTIOUS PUSTULES IN NEONATE

- - - -- - - - - - - -

Er thema Toxicum Transient Neonatal Pustular Melanosis

Starts on the second day of life · Presents at the time of birth
Palms and soles spared Occurs anywhere on the body
Erythema seen No erythema
Eosinophils presen.:..:t_______ _ _ _ _ _ _ ___,· Neutrophils present

Port wine stain - sturg weber, U/L not Salmon patch - physiological, crosses
crossing midline, reciuires RX midline . B/L

i~
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Hand, Foot and Mouth Disease

Caused by Coxsackie virus A16
No lymphadenopathy
No Pruritis

Vaccination in Immune Deficiency

I

....
Pnma,ya tpqpllacvto l'rimMy

~-
il«lffl

• Xlinbd • a.-. • Ominic

.,._
. • Seftre
Ca.non mtabirtad
-w.i.
..._...
lrnrnunod-4ide

,...,...,_,..,_
MY

AIIMIN cl«W~ AllliwwMO<W AIIIMIN awi;at~ N o,-d,wh

c:anttalndlau,,d
I

VAC CINATION IN PATIENTS RECEIVING STEROIDS

Receiving corticosteroids Receiving corticosteroids Receiving lower doses or

~2mg/kg/day or~ 20mg/day ~2mg/kg/day or~ 20mg/day receiving topical or inhalation
of prednisone ~14 days of prednisone < 14 days therapy
Live vaccines are given after Live vaccines are given after Live vaccines are given ·while
4 weeks of therapy 2 weeks of therapy receiving therapy

J UVENILE IDIOPATHIC ARTHRITIS

~~- - -- - - -- ---- ~-

Oligoarthritis (40- Polyarthritis Systemic arthritis Psoriatic arthritis

50%) Enthesitis related
arthritis
Involves </= 4 Involves > 4 joints Arthritis in >/= 1 joints
joints RF Negative (20- Hepatosplenomegaly
ANA positive 35%) Generalized lymph node
Anterior uveitis RF Positive < 10% enlargement
Serosistis
Skin rash
Presence of fever for at
least 2 week

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 Child Presenting with Polyarthritis
I
"'
:i> 6WNb

Histofy of SOfe throat

• Rheumatic f4Mff
•
WithffMK
•
•
Without fJN et
Rf MB,aliw poly JIA
• Post stteptoma.al rN<lM mhritis • Rf positive pofy .IA
Skin Rash Pr-esent

• HSP
lnanHd White cell counts white cell counu
lnanHd ESR Increased ESR
Systemic juvenile id~thk:.mritis Systemic lupus ~tom

REFRACTORY RICKETS

Not treated by cholecalcif erol

- - - - - - - - - ---- ---- -

Low calcium Normal Phosphorus Normal PTH

Increased PTH Normal PTH Low Phosphorous
Chronic renal failure Distal renal tubular acidosis · Fanconi syndrome
Vit amin D dependent r icket s 1A FGF 23 excess
Vitam in D dependent rickets 1B X-linked hypophosphatemic
Vitamin D dependent rickets r ickets
McCune Af br.ight syndrome
Tumour induced-Fibroma
Heman

CAUSES OF DRUG INDUCED RICKETS AND OSTEOMAL A CIA

- -

Cytochrome P450 Inhibition of 1 a Renal tubular defects Inhibition of osteoid '

enzyme Inducers hydroxylase (Fanconi syndrome) Mineralization :
(increased Ketoconazole Nucleotide reverse Alum inum
catabo lism of 25 Transcr iptase
hydroxy vitamin D) inhibitors
Phenytoin Tenofovir
Phenobarbitone Adelovir
Carbamazepine Cidofovir
Inh ibits enzyme Ritonavir
activit val roate

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Moderate Acute : Severe Acute

Malnutrition (MAM) Malnutrition (SAM)
Weight for Height
Mid upper arm
Circumference

O BESITY

Syndrome Obesity Endocrine Obesity '

Prader-Willi Syndrome Cushing Syndrome
Beckwith-Wiedemann Syndrome • Hypothyroidism
Pseudoh o arathyroidism
5-2-1-0 prophylaxis for obesity

5 fruits and vegetables

2 reduce screen time
1 hr of physical activity
0 sweetened drinks

MILESTONES

9 months Can stand with sue_port

2yrs Kickinq a ball
'
3 years Stand on i leq '
4yrs HopJ:>ing
5yrs Skipping

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