PEDIATRICS Congenital Heart Defects A. Congenital heart defects in general 1.

Etiology: usually not known - associated with maternal factors: a. infection b. alcoholism c. age over 40 years d. diabetes mellitus, type one e. genetics, chromosomal changes 2. General findings of congenital heart defects a. child small for age b. physiological failure to thrive c. exercise intolerance d. dyspnea while feeding e. squatting position f. clubbing of fingers 3. Physical consequences of cardiac problems a. increased workload; pulmonary hypertension; decreased systemic output; cyanotic defects b. can lead to hypoxemia and polycythemia c. concern: formation of thrombus with embolus Acyanotic defect - infant/child is "pink" 1. Pathology: hole in the heart's internal wall a. blood flows from heart's arterial (left) to venous (right) side or a "left to right shunt", not systemically, but only within heart itself b. size of defect will determine severity of condition 2. Four common types: all with increased pulmonary blood flow

B.

C.

Cyanotic defect: infant/child is "blue" 1. Pathology a. unoxygenated blood mixes with oxygenated, via a "right to left shunt" b. decreases oxygenation to the entire system c. results far more severe than acyanotic d. thrombus formation is always a concern

D.

Nursing care of the child with a congenital heart defect 1. Emotional, physiological, and psychological interventions 2. Assisting the child and family to adjust to special needs 3. Goals of treatment a. child will maintain adequate oxygenation and physiological stability b. family will understand signs and symptoms of the condition, and how to manage each of them c. child will attain milestones of normal growth and development d. when child has surgery, child and family will be prepared, know prognosis, and know how to give home care afterward 4. Nursing interventions

a.
b. c. d. e. f. g. h. i.

recognize CHF monitor height, weight, vital signs, pulses, pulse oximeter, intake and output give medications: digoxin (Lanoxin), furosemide (Lasix) or chlorothiazide (Diuril); monitor for desired outcomes and side effects recognize and treat pain appropriately: pharmacological and nonpharmacological interventions maintain a safe environment conserve energy maintain proper nutrition, with small, frequent feedings support and discuss treatment with parents place in proper position - slanting position with head elevated, older babies in infant seats, occasional knee-chest

Acquired Cardiovascular Disorders E. Hyperlipidemia: excessive lipids 1. Etiology: dietary, heredity 2. Pathophysiology a. increased lipids and cholesterol b. causes atherosclerosis, leading to coronary heart disease 3. Diagnosis: lab tests: increased LDL, lipids and cholesterol; decreased HDL 4. Management a. diet: ADA diet in two steps: i. < 30 kcal from fat; < ten from saturated fat; < 300 mg/L cholesterol ii. < 30 kcal from fat, < seven from saturated fat; < 200 mg/L cholesterol b. medications: colestipol (Colestid), niacin (Nicor), cholestyramine (Questran)

General

A.

B.

C.

Respiratory infections (illustration ) 1. Etiology: bacterial, viral; often influenced by age, season, preexisting disorder, living conditions 2. Findings: increased respiratory and heart rate, fever, nausea/vomiting, nasal discharge and blockage, mucus production, coughing, adventitious lung sounds 3. Nursing care goals: a. child will not exhibit findings of respiratory distress, will be able to clear secretions, and remain comfortable with a patent airway b. child will not spread infection to others c. child will ingest adequate fluids, and maintain hydration 4. Management: a. medications: antibiotics, antipyretics b. possible: anti-inflammatory, anti-mucolytics, bronchodilators, oxygen as needed c. chest physiotherapy d. nutrition and fluids Respiratory failure: inability to maintain adequate oxygenation 1. Predisposing factors a. obstructive anomalies, aspiration b. infections, tumors, anaphylaxis c. restrictive conditions: respiratory distress, cystic fibrosis, pneumonia, pneumothorax d. paralytic conditions 2. Findings a. restlessness, mood changes b. changes in LOC c. increasing rates of respiration and pulse d. dyspnea 3. Management a. frequent observation and physical exams, with pulse oximeter b. correct hypoxemia, maintain ventilation and deliver oxygen c. monitor for side effects and expected outcomes of therapy Airway obstruction and basic life support 1. Cardiac arrest is usually due to prolonged hypoxemia secondary to inadequate ventilation, oxygen or circulation 2. When following guidelines for pediatric life support, consider not just the child's age, but his or her size. Individual anatomy and development will vary. 3. Airway clearance techniques a. determine conscious versus unconscious child b. for infants and toddlers: back blows and chest thrusts c. for preschool and school-age: modified Heimlich maneuver ("astride")

Infant Respiratory Disorders D. Respiratory distress syndrome ( RDS): "hyaline membrane disease" 1. Etiology a. premature infants: usually due to surfactant deficiency b. newborns: birth asphyxia, multiple gestations, diabetic mother c. older children: trauma, drowning 2. Pathophysiology a. decrease in amount and/or quality of pulmonary surfactant b. in older children, surfactant may be washed out by drowning or fluid aspiration

3.

4.

Findings a. tachypnea, increased respiratory effort b. paradoxic "seesaw" respirations c. nasal flaring d. substernal retractions e. expiratory grunt, possible apnea f. cyanosis g. hypoxia Diagnostics a. physical exam, pulse oximeter

5.

6.

b. serum: ABG's, glucose, calcium c. chest radiograph confirmation Management a. oxygen therapy (possible mechanical ventilation) and chest physiotherapy b. possible medications: surfactant, prophylactic antibiotics, diuretics, inotropes, methyl-xanthines Concerns a. increased alveolar surface tension

E.

F.

impaired gas exchange (illustration 1 illustration 2 ) c. increased pulmonary resistance d. hypoperfusion Bronchopulmonary dysplasia: (BPD), chronic obstructive lung disorder 1. Etiology - at risk: a. infants requiring oxygen and/or lengthy mechanical ventilation b. infants surviving RDS c. lung immaturity 2. Pathophysiology a. mechanical ventilation presses lung tissue b. bronchial epithelium is damaged c. products of inflammation introduced, alveolar walls become thick, fibrotic d. continued mechanical ventilation affects the growth of new cells and paralyzes cilia e. lungs develop cystic areas (sacs) and atelectasis (collapsed alveoli); mucus moves less 3. Findings a. tachycardia and tachypnea b. increased work of breathing c. pallor d. cyanosis with activity e. restlessness 4. Management a. oxygen (possible mechanical ventilation) b. medications: diuretics, bronchodilators, anti-inflammatory agents in gradually decreasing amounts c. diet: increased calorie formulas and hydration Apnea: cessation of breathing for over 20 seconds 1. Etiologies a. prematurity b. foreign-body aspiration, drowning, or trauma c. incorrect positioning d. gastroesophageal reflux e. infections f. seizure g. hypoglycemia 2. Pathophysiology: dependent on type of apnea: a. central - absence of respiratory effort and air movement b. obstructive - respiratory effort but no air movement c. mixed - first central, then obstructive 3. Findings a. depend on type (above) b. color changes, hypotonia 4. Diagnostics: laboratory tests, chest x-rays, EEG, ECG, pneumocardiography, upper GI series 5. Management a. home apnea monitoring and basic life support (BLS) training to family b. medications: based on type and underlying condition

b.

Upper Respiratory Conditions

G.

Upper respiratory tract infections (URI) (illustration ) 1. Etiology: often acute viral nasopharyngitis, or the "common cold" 2. Pathophysiology

3. 4.

a. organism invades mucous membranes b. edema, vasodilation and increased mucus production c. usually self-limiting Findings: nasal congestion, sneezing, colored nasal discharge, low grade fever, cough, irritability Management: a. pharmacologic: antipyretics, decongestants (oral or nasal), analgesics b. cool mist humidifier c. adequate fluids d. rest

H.

I.

Sinusitis (illustration ) 1. Etiology: viral, bacterial, URI, obstructive deformities, cystic fibrosis 2. Findings a. cold that does not improve b. chronic nasal congestion, purulent nasal discharge c. headache d. tenderness over sinus areas e. halitosis 3. Diagnostics: sinus x-rays, CT 4. Management: antibiotics, antipyretics, analgesics, decongestants, antihistamines Acute pharyngitis 1. Etiology: streptococcus (bacterial); or viral (often accompanies tonsillitis) 2. Findings a. bacterial 1. "sicker" with sudden onset 2. very sore throat 3. high fever 4. headache b. viral 1. gradual onset 2. fever 3. cough, rhinitis 3. Diagnostics a. throat culture b. rapid strep 4. Management: medications a. penicillin for strep b. possible antipyretics and analgesics

J.

Tonsillitis 1. Etiology: bacterial, viral in association with pharyngitis 2. Pathophysiology: infection and inflammation enlarge tonsils; as airway narrows, it hinders swallowing and breathing 3. Findings a. "kissing tonsils" b. sore throat c. halitosis d. mouth breathing; snores at night e. fever 4. Diagnostics: history, physical exam, throat culture 5. Management a. supportive: antibiotics, fluids, rest, antipyretics, analgesics b. surgery - tonsillectomy 6. Concern: teach parents that, post tonsillectomy for several days, child needs quiet activity, monitoring for bleeding, antipyretics and analgesics (no aspirin). 7. Adenoidectomy may be done with tonsillectomy, if adenoiditis is present Croup syndromes (including laryngitis, tracheitis, epiglottitis) )

K.

1.

Definition: several airway-blocking infections, common in children (illustration a. signs of croup: 1. inspiratory stridor

b.

2. harsh/brassy cough, barking cough 3. hoarse voice 4. respiratory distress types, by primary area affected: 1. subglottal area: acute spasmodic croup, laryngitis, laryngotracheobronchitis (LTB), tracheitis

2.

3. 4.

2. supraglottal area: epiglottitis (illustration ) Etiology a. usually viral b. occasionally bacterial (tracheitis, epiglottitis) c. younger children with "true croup" (spasmodic croup) d. older children with tracheitis and epiglottitis Pathophysiology: mucosa inflamed; edema narrows the airway Findings a. classic: "barky" harsh cough, stridor, hoarseness,fever, purulent secretions, dyspnea if severe b. bacterial: child looks "sicker" c. epiglottitis manifests the four "D's" 1. drooling 2. dysphagia (difficulty swallowing) 3. dysphonia (hoarse voice) 4. distressed inspiratory efforts

Similarities And Differences Of Diseases Included In The Croup Syndromes

5.

6.

Management a. viral i. cool air/mist; fluids ii. if inpatient, nebulized racemic epinephrine and inhaled steroids iii. antipyretics b. bacterial: same as above with antibiotics, possible intubation c. concerns: if you suspect epiglottitis, never attempt to directly visualize epiglottis with tongue depressor; it could precipitate laryngospasm. d. epiglottitis is a medical emergency; tracheotomy may be necessary Nursing intervention a. teach parent and child signs of impending airway obstruction b. report increased pulse, respirations, retractions, increased restlessness

Lower Airway Disorders L. Bronchiolitis: acute infection at bronchiolar level 1. Etiology a. viral: RSV (respiratory syncytial virus) most common b. occasionally bacterial 2. Pathophysiology a. virus spreads via direct contact

b. c.

d.
3. 4. 5.

enters body via nose or eye leads to edema, mucus accumulation and cellular debris which obstruct bronchioles can progress to atelectasis

Findings a. usually mild URI b. sneezing, productive cough, low-grade fever, nasal discharge, adventitious lung sounds Diagnostics: history, RSV/viral nasal washing, chest x-ray Management depends on severity a. mild: fluids, humidification, rest b. severe: antiviral, IV fluids, possibly bronchodilators, steroids and mechanical ventilation. Maintain contact secretion precautions. c. prophylaxis: respiratory syncytial virus immune globulin in high risk infants Pneumonia: inflammation of lung parenchyma (illustration ) a. Etiology: usually classified by anatomic distribution or pathogen A. most commonly viral B. sometimes bacterial C. sometimes aspiration of foreign substance b. Pathophysiology A. triggers terminal airways, alveoli infiltrate and cell destruction B. cellular debris falls into lumen C. bacterial agent can reach circulatory system via pulmonary lymphatics c. Findings A. can be abrupt or insidious B. adventitious lung sounds, fever, malaise, nonproductive cough progressing to more severe with retractions, respiratory distress, productive cough d. Management depends on type A. viral - oxygen, chest physiotherapy, fluids B. bacterial - antibiotics, oxygen, chest physiotherapy, fluids C. aspiration - supportive therapy, treatment of secondary complication Aspiration of foreign body a. Etiology: child aspirates solids, liquids, vegetative matter into air passages, most common in older infants and children up to three years of age b. Pathophysiology: most substances become lodged in bronchi, and severity is determined by location, substance aspirated and extent of obstruction c. Findings: sudden coughing, gagging, wheezing, cyanosis, dyspnea, and stridor d. Diagnostics: chest x-ray, fluoroscopy, bronchoscopy e. Management: direct laryngoscopy or bronchoscopy to remove object, then supportive therapy Asthma/reactive airway disease a. Definition: chronic inflammatory disorder, manifested by periods of exacerbations and remissions; has an allergic component b. Etiology A. genetic predisposition B. triggers are: allergens, infection, stress, exercise, medical conditions, medications. C. types: intrinsic, extrinsic, occupational

6.

C.

7.

8.

c.

Pathophysiology (illustration ) A. trigger leads to an immediate phase reaction (cell activated, with mast cell, eosinophils and histamine released with other mediators of inflammation) B. resulting in bronchoconstriction with additional granulocyte response with more inflammatory presence (illustration ) C. later phase reaction (additional inflammation and hyperresponsiveness) Findings A. classic: hacking cough, wheeze on expirations, dyspnea B. cough may be nonproductive at first, to productive with mucus C. change in LOC, restlessness Diagnostics A. physical exam, history B. pulmonary function tests (PFT)

d.

e.

9.

C. bronchodilator treatments, chest x-ray, skin testing, CBC with differential Management A. goals: normal growth and development, patent airway, good control B. preventive: allergen control and avoidance C. use of peak flow meter D. medications A. long term control (preventor) medications: to achieve and maintain control of inflammation; also called controllers A. corticosteroids B. cromolyn sodium C. nedocromil D. long acting beta adregenics E. methylxanthines F. leukotriene modifiers B. quick relief (rescue) medications to treat acute symptoms and exacerbations: A. short acting beta adrenergics B. anticholinergics C. systemic corticosteroids g. Acute management A. use of bronchodilators B. steroids (inhaled, IV and/or oral) C. oxygen, IV fluids, possibility of intubation h. Concerns A. child and family must comply with medications and treatments B. use of metered-dose inhaler (MDI) with a spacer C. risks include overuse of bronchodilators i. Complications A. exercise-induced bronchospasm B. status asthmaticus Increased intracranial pressure (ICP) a. Etiology A. congenital or acquired (from trauma, lesion, infection) B. also see Pediatric Oncology (See section XI of this lesson) b. Pathophysiology: swelling caused by irritation or bleeding into brain tissue c. Findings A. infant: bulging fontanels, widened cranial sutures, high-pitched crying, irritable f.

(illustration ) B. child: headache, nausea, vomiting, lethargy, diplopia, seizures d. Management A. principle of maintaining cerebral perfusion pressure (CPP), which equals mean arterial blood pressure minus ICP B. medications: osmotic diuretics, antihypertensives, anti-seizure C. raise head of bed D. mechanical ventilation, oxygen E. in severe cases, ICP monitor F. minimize external stimuli 10. Hydrocephalus a. Etiology: congenital, acquired, or idiopathic b. Pathophysiology: depends on type A. communicating: impaired absorption of CSF within the subarachoid space B. c. (illustration ) noncommunicating: obstruction of the flow of CSF through the ventricular system

Findings A. changes from baseline neurological status, and often same as increased ICP B. infant (signs of increased intracranial pressure) A. bulging fontanels, increased head circumference B. "setting sun" eyes, pupils slow to constrict to light C. eats poorly D. high-pitched cry E. variable pulse, changes in respirations

C.

older child (signs of increased intracranial pressure) A. headaches B. dizziness C. vomiting D. diplopia

d.

e. 11. Seizures a. Definition: brief malfunctions of the brain's electrical system that result from cortical neuronal discharge b. Etiology: cerebral, biochemical, post-traumatic, febrile, idiopathic, most common neurologic dysfunction in children c. Pathophysiology A. trigger electrical discharges affecting nearby normal cells B. spreads throughout brain reaching midbrain reticular regions C. possible generalized seizure with neurological symptoms d. Findings A. determined by site of origin B. may include altered levels of consciousness, involuntary movements and changes in perception C. seizure may be a finite event, with limited manifestations D. may have an aura E. status epilepticus is continuous seizure lasting more than 30 minutes or a series of seizures from which the child does not regain a premorbid level of consciousness F. febrile seizures: occur in association with a fever, usually greater than 38.8 degrees Celsius (101.8 degrees Fahrenheit) G. may be nonrecurrent (acute) such as febrile episode or recurrent (chronic) such as epilepsy D. Epilepsy 1. Definition: chronic seizure disorder with recurrent & unprovoked seizures. 2. Etiology: unknown. international classification: partial and generalized a. partial: limited to a particular local area of the brain b. generalized: involves both hemispheres of the brain 1. Pathophysiology: see seizures Management see seizure precautions, seizure care, postictal care a. supportive i. airway: suction, oxygen ii. safety: loosen clothing, place on floor, clear area, remove restrictive clothing b. labs: toxicology screen, ABG's, electrolytes, anticonvulsant drug level, possible c. d. E. spinal tap findings (illustration ) pharmacology - antiepileptic drugs goal: to control seizures and/or reduce frequency

E. ataxia Management A. surgical placement of ventriculoperitoneal (VP) shunt in neonates, infants, and older children B. monitor for increased intracranial pressure C. measure head circumference Complications: shunt infection and malfunction

2.

Neural tube defects (NTDs) 1. Anencephaly a. most serious b. both cerebral hemispheres are absent c. condition is incompatible with life 2. Spina bifida (SB) a. etiology: incomplete closure of vertebrae and neural tube; unknown cause b. pathophysiology i. spina bifida occulta: defect not visible externally

ii.

spina bifida cystica: visible defect with an external sack-like protrusion meningocele: encases meninges and spinal fluid but no neural elements; no neurological deficit meningomyelocele: contains meninges, spinal fluid, and nerves; varying neurological deficit

a. b.

F.

Cerebral palsy (CP) 1. Etiology a. disorders characterized by early onset and impaired movement and posture; non-progressive b. cause: abnormality in extrapyramidal or pyramidal motor system (cortex, basal ganglia, cerebellum) c. can occur prenatally, perinatally, or postnatally d. may be accompanied by perceptual problems, language deficits and intellectual involvement 2. Pathophysiology

findings vary widely according to degree of spinal defect management: i. initial care protect sac from rupturing and drying out moist sterile nonadherent dressing fluids positioning early surgical closure of lesion ii. long term protect skin integrity maintain bladder & bowel function support musculoskeletal integrity iii. complications: hydrocephalus after surgical repair; may require VP shunt meningitis urinary tract infection pneumonia

a.
b. 3.

G.

c. Findings a. primary disturbances: abnormal muscle tone and coordination b. spastic movement in one or more extremity c. athetoid movements d. primitive reflexes persist e. disturbances in gait, abnormal posture f. impairments in speech and swallowing 4. Diagnostics: physical exam, EEG, CT 5. Management a. physiological: nutrition, musculoskeletal, respiratory, integumentary b. cognitive Down syndrome (trisomy 21) 1. Etiology: extra group G chromosome, chromosome 21. Associated with late maternal age 2. Pathophysiology: depends on which region of chromosome 21 was altered a. distinctive facial features b. heart defects c. mental retardation (varies from severe retardation to low average intelligence) d. dermatologic changes e. incomplete embryogenesis 3. Manifestations: a. facial features i. brachycephaly

classifications: athetoid, spastic, ataxic, mixed associated defects: mental retardation and cognitive impairment, hearing or visual losses, attention deficit disorder (ADD) high metabolic rate, leading to increased calorie expenditure

b.

ii. flat occiput iii. protruding tongue iv. high-arch palate v. almond shaped eyes vi. low slung ears body features

H.

i. short, broad hands with simian crease (illustration ) ii. short, broad neck iii. dry skin iv. large space between great toe and second toe 4. Management a. supportive: specific to body system affected b. promotion of developmental progress c. cognitive Werdnig-Hoffmann syndrome ("floppy infant" syndrome) 1. Etiology: inherited autosomal-recessive disorder 2. Pathophysiology: a. skeletal muscles atrophy b. arises form pathology of anterior horn cells of spinal cord brain stem and motor nuclei of brain stem 3. Clinical manifestation: variable onset a. earlier onset, increased severity. b. weakness, legs externally rotated, abducted and flexed at hips c. limited range of arm movement d. weak cry 4. Diagnostics: electromyography, physical exam 5. Management: supportive, with emphasis on respiratory and musculoskeletal systems 6. Prognosis: early death

Infectious Neurological Disorders I. Guillain-Barre syndrome - infectious demyelinating polyneuritis 1. Etiology: unknown - possible sequela to microbial infection or administration of vaccines 2. Pathophysiology a. an unknown factor inflames spinal and cranial nerves b. inflammation compresses and demyelinates nerves c. nerves conduct impulses poorly resulting in paralysis of muscles innervated by involved nerves 3. (illustration ) Findings a. preceded by mild influenza-like illness or sore throat b. gradual progression of symptoms c. muscle tenderness, paresthesia usually ascend from feet/legs upward progressing to total paralysis Management a. short-term: supportive and symptomatic b. based on physiological needs and body system affected: possible tracheostomy, ventilatory support, IV fluids c. long term: integumentary, musculoskeletal expiratory, respiratory systems Prognosis a. death occurs from respiratory failure b. better outcomes for younger ages, no requirement for ventilatory assistance, slower progression, normal peripheral nerve function

4.

5.

J.

Botulism 1. Etiology a. food poisoning from anaerobic bacillus: clostridium botulisum b. three types: infantile, classic, wound 2. Findings a. usually appear within 12 to 36 hours b. blurred vision, diplopia, lethargy, vomiting and dysphagia 3. Management a. supportive: dependent on body system affected

K.

b. protect ventilation, respiration, and nutrition Reye's syndrome (toxic encephalopathy) 1. Etiology: virus, drugs (salicylate association), toxins, genetics 2. Findings a. related to cerebral edema and fatty changes in liver b. viral upper respiratory infection c. fever d. severe nausea and vomiting e. profoundly impaired consciousness f. liver dysfunction g. coma 3. Diagnostics: liver biopsy 4. Management a. supportive: dependent on body system affected b. avoid asprin and other salicylates in children with viral diseases such as influenza or varicella disease

Traumatic Neurological Conditions Head trauma 1. Etiology: force to the scalp, skull, meninges, or brain 2. Pathophysiology a. directly related to force of impact and secondary forces b. types: primary and secondary c. cerebral function depends on oxygen, glucose and blood flow to brain; cerebral edema and hypoxia can occur quickly 3. Findings: neurological status depends on ICP 4. Management a. acute i. control ICP: medications, ventilatory support, positioning, fluids ii. surgical repair of fractures b. long-term rehabilitation 5. Complications: hemorrhage, infection, cerebral edema and herniation through the tentorium a. epidural hemorrhage b. subdural hemorrhage The endocrine system L. Three types of tissues 1. cells that send chemical message via hormones 2. target cells in end organ that receive chemical message 3. environment: medium through which chemical message travels from the site of synthesis to area of cellular action M. Endocrine function 1. endocrine + autonomic systems = neuroendocrine system 2. neuroendocrine system: synthesizes and releases chemical substances that then regulate bodily function: messages are carried by nerve impulses in the nervous component and by blood in the endocrine system. (illustration ) the two systems function synergistically. Impulses transmitted via the nervous system stimulate the hypothalamus to secrete releasing or inhibiting factors Hypopituitarism 1. Definition: diminished or deficient secretion of pituitary hormones 2. Etiology: tumors, hypoplasia, auto-immune, congenital, acquired, irradiation, malnutrition, trauma 3. Pathophysiology: leads to gonadotropin deficiency 4. Findings a. usually dependent on hormone involved and age of onset b. absence or regression of secondary sex characteristics c. example: slow growth, short stature, normal intelligence; delayed puberty 5. Management a. depends on cause b. hormone replacement therapy: growth hormone "Precocious puberty" 3. L.

2.

N.

O.

P.

Definition: manifestations of sexual development before age nine in boys or age eight in girls 2. Etiology: brain lesions, inflammatory disorders, idiopathic 3. Pathophysiology a. premature activation of hypothalamic-pituitary-gonadal axis b. early increased release of LH and FSH c. early acceleration of linear growth with early closure of growth plates d. ultimate height less than if puberty had been normal 4. Findings a. development of breasts in prepubertal females b. early development of sexual hair c. isolated menses d. development of secondary sex characteristics 5. Management a. directed toward specific cause b. hypothalamic - pituitary origin: luteinizing hormone - releasing hormone (LHRH) injections monthly SIADH: syndrome of inappropriate antidiuretic hormone 1. Definition: hypersecretion of antidiuretic hormone or ADH (vasopressin) 2. Etiology: infections, head trauma, or brain tumors 3. Pathophysiology a. kidneys reabsorb too much free water b. decreased urine output, increased specific gravity (SG), and decreased serum sodium 4. Findings a. urine SG > 1.030 b. anorexia, nausea, weakness, stomach cramps c. increased weight (edema) d. decreased serum sodium 5. Management a. restrict fluids to correct hyponatremia b. correct underlying etiology c. monitor I & O, electrolytes, neuro status d. observe for signs of fluid overload Hypothyroidism (illustration ) 1. Definition: deficiency of thyroid hormone (TH); common childhood endocrine disorder 2. Etiology a. congenital or acquired deficiency in thyroid hormones b. thyroid irradiation 3. Pathophysiology a. absent or underdeveloped gland b. decreased triiodothyronine (T3) or thyroxine (T4) 4. Findings: depend on extent of dysfunction and age of child at onset a. lethargy, constipation, feeding problems b. hypotonia, large tongue c. dry skin, weight gain, puffy eyes, sparse hair d. intolerance to cold e. slowed growth, developmental delay and/or retardation if T4 low at birth and thyroid replacement not started f. if findings develop after two to three years, when brain has grown, no mental retardation 5. Diagnostics: a. history and physical b. increased TSH c. decreased serum T3 and T4 d. anemia e. decreased BMR (basal metabolic rate) f. increased cholesterol and triglycerides g. hypoglycemia 6. Management: a. lifelong hormone replacement (levothyroxine) b. rest c. protect client from cold

1.

Q.

R.

S.

T.

U. V.

Hyperthyroid: "Graves' disease" 1. Definition: hypersecretion of thyroid hormones, causing increased BMR or hyperactivity of thyroid gland 2. Etiology: auto-immune response to TSH (thyroid-stimulating hormone) receptors; idiopathic; familial 3. Findings a. increases in: BMR, appetite, nervousness, heart rate b. gradual weight loss despite voracious appetite c. lowered tolerance to heat d. exophthalmos (eyes bulge) e. myopathy f. personality changes, poor school performance, mood instability g. linear growth and bone age accelerated h. insomnia i. increased blood pressure 4. Diagnostics a. history and physical b. palpable thyroid enlargement (goiter) c. elevated serum T3 and T4 levels d. elevated radioactive iodine uptake e. presence of thyroid antibodies f. decreased TSH levels 5. Management a. antithyroid therapy b. surgery (subtotal thyroidectomy) 6. Concern: thyrotoxicosis or "thyroid storm" from sudden release of hormone: a. can be life threatening b. findings i. acute onset of severe irritability ii. vomiting iii. diarrhea iv. hyperthermia v. hypertension vi. tachycardia vii. prostration c. treat with: cooling blanket d. propranolol (inderol), potassium iodine (thyro-block) e. antithyroid drugs Diabetes mellitus - type 1 1. Definition: deficiency of hormone insulin 2. Etiology: genetic, auto-immune response; usually with viral trigger 3. Pathophysiology: a. trigger directs islet cell antibodies against cell surfaces b. antibodies destroy the insulin-secreting Beta cells c. less insulin means glucose is blocked from intracellular space d. when glucose level exceeds kidney's threshold (about 180 mg/dl), result is hyperglycemia e. kidney then "spills" glucose into urine, producing osmotic diuresis f. starved for glucose, body instead breaks down fats, producing ketones 4. Findings a. hyperglycemia: i. the three "polys" of diabetes: polydipsia, polyuria, polyphagia ii. additional findings: fatigue, hunger, weight loss, enuresis Management 1. medications: insulin (regular, NPH); glucagon (hypoglycemic emergency only) 2. other: diet (carbohydrate counting, age-appropriate), activity levels 3. blood glucose monitoring Concerns: "honeymoon period" and insulin regulation, compliance, sick day management, ketones Complications: 1. DKA (diabetic ketoacidosis) or extreme hyperglycemia (blood sugar > 350 mg/dl) a. etiology: not enough or no insulin b. findings i. fruity breath, decreased level of consciousness

ii. iii. iv.

v.
2.

nausea/vomiting, abdominal pain 10 percent dehydration increased urine output Kussmaul's respirations

management a. fluids; control the level of serum osmolality b. regular insulin (IV drips and then subcutaneous) c. frequent monitoring of blood glucose d. frequent monitoring of electrolytes; neuro checks W. Hypoglycemia: 1. etiology: most common occurrence of insulin therapy and bursts of physical activity, without additional food or with missed meals. 2. findings a. fatigue b. nervousness c. pallor d. sweating e. palpitations f. funger g. loss of coordination h. seizures i. coma 3. management a. 10 to 15 mg of simple carbohydrate b. followed by complex carbohydrate such as slice of bread or crackers c. occasionally glucagon is prescribed

X.

Dehydration (illustration ) 1. Occurs when total output of fluid exceeds total intake 2. Compared to adults, children a. are less able to concentrate urine b. as infants, they have immature kidney and immune-regulatory systems c. have a higher metabolic rate d. have more body surface in relation to body mass e. because more of body weight is fluid, children need more fluid and lose more urine per Kg of body weight 3. Types of dehydration (serum sodium determines type) a. isotonic: occurs in conditions in which electrolyte and water deficits occur in balanced proportions b. hypotonic: occurs in conditions in which electrolyte deficit exceeds the water deficit c. hypertonic: occurs in conditions in which water loss exceeds electrolyte loss 4. Pathophysiology a. decreased fluids and electrolytes from ECF (extracellular fluid) b. leads to eventual loss of fluid from ICF (intracellular fluid) c. cellular dysfunction, shock 5. Complications a. fluid losses: monitor urine output and specific gravity b. electrolyte losses: monitor sodium, potassium, chloride, calcium c. acid-base disturbance: metabolic acidosis 6. Findings of dehydration

7.

Management of pediatric dehydration a. monitor the 4 first signs and 3 second signs of dehydration b. administer fluids (oral, NG, IV)

Monitoring the Dehydrated Child Dehydration means not enough fluids. 4 First Signs: Less skin turgor Lower weight Less urine output Altered electrolytes And dehydration quickly affects three systems: cardiac, respiratory and neuro. So monitor for 3 second signs: Heart rate increases Respiratory rate increases Level of consciousness and awareness diminished B. Edema 1. 2.

Definition: excess fluid swells interstitial tissues Etiology a. defect in homeostasis b. renal disease; liver disease; nephrotic syndrome c. starvation d. burns e. decreased colloidal oncotic (swelling) pressure

3.

Pathophysiology: increased venous pressure - increased capillary permeability, decreased plasma proteins; contributing to the edema may be obstructed lymph flow or sodium retention a. findings: pitting edema, increased heart rate i. can be localized or generalized swelling ii. pitting edema - more common with localized iii. increased heart rate b. management - treat underlying cause

C.

Vomiting 1. Definition: forceful ejection of gastric contents through the mouth 2. Etiology: infection, obstruction, allergy, psychological causes, motion sickness, neurologic lesions 3. Pathophysiology a. cause stimulates emetic center of brain b. mechanism of vomiting involves antonomic nervous system: i. salivation, sweating ii. pallor, increased heart rate iii. contraction of stomach antrum & duodenum c. types of vomiting: regurgitation, forceful, projectile 4. Management a. detect and treat the underlying cause b. prevent dehydration, electrolyte loss and acid-base disturbance c. provide anti-emetic medications

Upper GI Disorders D. Tracheoesophageal fistula (TEF) and esophageal atresia (EA) 1. Definition: failure of esophagus to develop as a continuous passage and a failure of the trachea and esophagus to separate into distinct structures 2. Etiology: congenital, idiopathic, VATER syndrome (combination of vertebral, anorectal, and renal abnormalities in addition to TEF) 3. Findings a. coughing b. cyanosis with feeds c. increased oral secretions d. depend on type of defect 4. Diagnostics: history, abdominal x-rays 5. Management a. pre-op: airway patency, NPO, IV therapy, positioning b. prevention of aspiration pneumonia c. surgery Gastroesophageal reflux disease: GERD (illustration ) 1. Etiology: physiological, functional, pathologic. Very common. If GERD occurs often and findings persist, treat it. 2. Pathophysiology: defect in neural control of lower esophageal sphincter; stomach contents flow back into esophagus 3. Findings a. vomiting or spitting up with meals b. recurrent aspiration pneumonia c. failure to thrive d. anemia e. for older child, heartburn f. heme-positive emesis or stools g. irritable h. pallor/cyanosis 4. Diagnostics: history, barium swallow, upper GI series, esophageal pH, manometry studies, endoscopy 5. Management a. depends on the severity of findings b. none if child is thriving without findings c. diet: thicken feeds; decrease caffeine, acid foods and solutions; give small, more frequent feeds with frequent burping d. position: prone with head of bed elevated, or flat prone following feeding and at night e. medications: H2-receptor antagonists, prokinetic agents

E.

F.

f. surgery: fundoplication 6. Concerns: airway, fluid and electrolyte balance, apnea Pyloric stenosis 1. Etiology a. unknown cause b. hypertrophy, hyperplasia of circular muscles of pylorus 2. Pathophysiology a. trigger irritates mucoid lining of pylorus; edema b. narrowed pylorus resists passing of fluid; chyme c. pylorus then enlarges and contracts with more force to attempt gastric emptying d. slowly pylorus constricts, and resistance persists until next cycle 3. Findings a. usually begins at two to four weeks of age b. progressive, projectile, nonbilious vomiting after eating c. metabolic alkalosis d. movable, palpable, firm, olive-shaped mass in right upper abdominal quadrant e. irritability, crying, hunger 4. Diagnostics: history, abdominal x-ray, upper GI, ultrasound 5. Management a. pre-op: IV fluids, NPO, surgery (pyloromyotomy) b. post-op: small, frequent feedings initially, gradually increasing

6.

Concerns: fluid and electrolyte balance, airway, nutrition

Lower GI Disorders G. Constipation 1. Definition: infrequent passage of firm or hard stools 2. Etiology a. triggered by diet, medication, dehydration, emotions, or neurogenic structural disorders (Hirschsprung's disease) (illustration ) c. systemic disorders (hypothyroid) Findings a. abdominal pain and cramping b. palpable, movable fecal mass c. malaise, anorexia, nausea Diagnostics: abdominal x-rays, rectal exam, palpation & percussion Management a. prevention (higher fiber diet, fluids, exercise, regular toileting habits) b. medications (stool softeners, enemas, laxatives)

b.

3.

4. 5. H.

I.

Diarrhea 1. Etiologies: intestinal infections (bacterial, viral, parasitic); food intolerance; overfeeding; medications; stress; malabsorption; colon disease 2. Pathophysiology: a. causative factor - irritates mucosal lining b. damages microvilli, increases secretion and decreases absorption c. abnormal intestinal water and electrolyte transport d. increased intestinal water permeability and dehydration in younger children. e. possible metabolic acidosis f. can be acute or chronic 3. Findings a. dehydration (mild, moderate, severe) b. increased stooling c. increased heart & respiratory rate d. dry hot skin e. dry mucous membranes f. decreased urine, decreased tearing in infants 4. Management a. goals: restore fluid and electrolyte balance and return bowel to normal functioning b. fluids: oral rehydration or parenteral rehydration c. medications: antibiotics, antidiarrheals Celiac disease = gluten-sensitive enteropathy (GSE) = celiac sprue 1. Etiology: absorption problem with genetic predisposition, possibly immune abnormality 2. Pathophysiology a. inability to digest gliadin (byproduct of gluten); permanent intolerance of gluten. b. increasing levels of glutamine in the intestine, toxic to mucosal cells atrophy of villi and decreased absorptive surface (illustration ) d. malabsorption of fats, carbohydrates, vitamins and electrolytes e. gluten is found in the grain of wheat, barley, rye, and oats Findings (most often appears between ages of one and five years) a. diarrhea (stools: pale and watery, offensive odor) b. abdominal distention c. failure to thrive d. vomiting e. muscle wasting f. steatorrhea g. anorexia h. abdominal pain Diagnostics: a. history (symptoms occur three to six months after infant begins eating grains) b. serum anti-gliadin antibody (AGA)

c.

3.

4.

5.

c. jejunal biopsy Management a. diet: gluten-free with vitamin supplements b. crisis: IV fluids, steroids

J.

K.

Intussusception: one of the most frequent causes of intestinal obstruction between ages of three months and five years 1. Etiology: unknown - possible hypertrophic response to virus 2. Pathophysiology a. trigger - bowel "telescopes" inside itself causing obstruction b. pressure on bowel leads to bleeding c. possible mesenteric ischemia d. edema and possible bowel necrosis, perforation, peritonitis, or shock e. most common site: ileocecal valve 3. Findings a. sudden acute abdominal pain b. bilious vomiting c. currant jelly stools d. sausage-shaped abdominal mass e. lethargy 4. Management a. priority goal: restore bowel to normal position and function quickly, and stabilize fluids b. non-surgical hydrostatic reduction (barium, water-soluble contrast, air pressure) c. if not successful, surgery 5. Concerns a. fluid status b. pain c. ischemia d. monitor for barium excretion Hirschsprung's disease 1. Definition / etiology: congenital aganglionic megacolon 2. Pathophysiology a. absence of autonomic (parasympathetic) ganglion cells, usually at rectum and part of large intestine b. intestine does not propel stool c. stool builds up; colon dilates, constipation results with risk of intestinal rupture 3. Findings a. newborn: failure to pass stool or merconium in first 24 hours of life, reluctance to ingest fluids, bile-stained vomitus, distended abdomen, "ribbon-like" stools flat, wide, and wavy b. later: failure to thrive, distended abdomen, constipation, signs of fecal impaction 4. Diagnostics: history and physical exam, radiographic barium enema, rectal biopsy, anorectal manometry 5. Management a. surgical correction: remove aganglionic portion b. temporary ostomy for three to six months, then reanastomose

Associate "Hir"schsprung's with a girl, "her," who wears "ribbons" in her hair - to recall that "ribbon-like" stools are a classic finding. B. Urinary tract infection 1. Definition a. bacteria in urine and inflammatory response b. may involve any structure in urinary system: kidney (pyelonephritis), ureters, bladder (cystitis) or urethra c. findings will point to location d. peak age: toddler to preschool age 2. Etiology

3. 4.

5. 6.

7.

a. bacterial, structural defect, physiological b. extrinsic (example: foley catheter, medications) c. improper perineal hygiene Pathophysiology a. organism usually ascends through urethra to bladder. b. with a structural defect, urine flows back from ureters into kidney. Findings often depend on age a. under two years: often nonspecific resembling GI disturbance i. failure to thrive, feeding problems, nausea/vomiting, ii. dysuria, persistent diaper rash, abdominal distention b. over two years: i. enuresis, daytime incontinence in toilet-trained, foul smelling urine ii. frequency and urgency, dysuria iii. possible pyelonephritis: similar signs but with fever, back pain, and lethargy Diagnostics: urine culture and serum testing, radiographic testing Management a. antimicrobials b. antipyretics c. analgesics d. fluids (IV and oral) e. education i. wipe front to back (in girls) ii. do not delay urination Concerns a. hydration b. recurrence c. long term complications such as scarring, renal failure

C.

Enuresis 1. Definition: inability to control bladder functions, often due to maturation; nocturnal, or "bed wetting" very common 2. Etiology/findings a. primary: never dry at night, and due to CNS or psychological reasons b. secondary: child has been toilet trained and becomes incontinent again i. findings as above ii. due to infection, medications, trauma 3. Diagnostics: history, urine tests, serum culture 4. Management a. primary i. have the child participate in activities ii. limit fluid at night, void before bedtime iii. imagery, behavioral conditioning 1. tricyclic antidepressant; imipramine 2. desmopressin (DDAVP) iv. secondary: treat underlying cause

Upper Urinary Tract Disorders D. Vesicourethral reflux (VUR) 1. Definition: retrograde flow of bladder urine into the ureters 2. Etiology a. primary (congenital anomaly) b. secondary (acquired, usually associated with UTI) 3. Pathophysiology a. bladder reflux b. residual urine from ureters remains in bladder until next void c. increases chance for and perpetuates infection d. vesicoureteral reflux grading system: grade one to five 4. Findings: UTI with chronic findings & recurrences 5. Diagnostics: radiographic studies 6. Management a. medications: for grades one to four, low-dose antibiotics; monitor b. surgery: for severe cases, grade four or grade five

E.

F.

Concerns: a. renal scarring b. all children in family should be screened Acute glomerulonephritis (AGN) 1. Etiology a. acute bacterial infection (pharyngitis, impetigo) b. underlying systemic disorder 2. Pathophysiology a. infection (usually group A B-Hemolytic strep) provokes immune complex response b. immune complexes trapped in glomerular capillary loop c. activate inflammatory response, which injures capillary walls d. decrease lumen functions and GFR (glomerular filtration rate) e. decreased filtration of plasma results in excessive acummulation of water and retention of sodium f. onset appears after latent period of about ten days 3. Findings a. oliguria b. edema (periorbital and peripheral) c. hematuria ("smoky" or "tea-colored" urine) d. mild hypertension e. lethargy f. moderate proteinuria g. loss of appetite 4. Diagnostics: urine testing, serum (antibody, complement, CRP, ESR, WBC), throat culture, history of antecedent strep infection 5. Management a. no specific treatments; recovery spontanteous and uneventful in most cases b. supportive with careful regulation of fluid balance; I & O, daily weights c. medications: antihypertensives, if needed d. possible diuretic nutrition (low in sodium, protein, potassium) 6. Concerns: hypertensive encephalopathy, cardiac decompensation, renal failure, edema and fluid overload Chronic nephrosis = nephrotic syndrome 1. Massive proteinuria, hypoalbuminemia, hyperlipemia and edema 2. Etiology: not fully understood - possibly renal lesions or other processes a. types: 1. primary (idiopathic nephrosis): restricted to glomerular injury 2. secondary: develops as part of systemic illness 3. Pathophysiology a. glomerular alteration and increased permeability to plasmaproteins, especially albumin b. plasma protein losses; increased presence in urine, decrease plasma volume, colloidal osmotic pressure in capillaries decreases c. hydrostatic pressure is greater than colloidal osmotic pressure resulting in fluid accumulation in interstitial spaces and body cavities d. shift in plasma fluid leads to hypovolemia e. hypovolemia - triggers kidneys to produce renin, and angiotensin which stimulates the release of aldosterone and increases the reabsorbtion of water and sodium f. aldosterone increases g. decreased blood pressure also causes release of ADH leading to increase in water absorption 4. Findings a. progressive weight gain b. puffiness of face c. generalized edema (insidious) d. periorbital edema e. loss of appetite f. oliguria g. lethargy h. pallor 5. Diagnostics: history, urine tests: massive proteinuria, serum testing 6. Management: mainly supportive

7.

G.

H.

a. diet: limit sodium, but high potassium and protein b. medications: corticosteroids, immunosuppressants, diuretics 7. Concerns: hypercoagulability and thrombosis, fluid excess, alterations in skin integrity, recurrence Hemolytic uremic syndrome (HUS) 1. Definition a. acute disorder shows the "triad": 1. hemolytic anemia 2. thrombocytopenia 3. acute renal failure b. occurs most often in children under four 2. Etiology: unknown; some association with genetics, endotoxins, bacteria (E. coli, rickettsia, coxsackie), enzyme deficiency, decreased antioxidants 3. Pathophysiology a. usually follows an acute GI or upper respiratory infection (URI) b. pathogen attaches to GU tract c. GU tract produces toxins that damage capillary walls d. inflammation - glomerular vessels less capable of filtration e. anemia occurs (due to RBC fragmentation) f. thrombocytopenia (due to platelets trapped in small vessels) 4. Findings a. prior URI b. rapid onset of pallor c. accompanying hemorrhagic manifestations such as bruising or rectal bleeding d. triad e. hypertension 5. Diagnostics: identify "triad" from history and lab testing 6. Management a. supportive b. for anemia: transfusions c. for renal symptoms: fluids, possible dialysis d. treatment of hypertension e. correction of acidosis and electrolyte disorders f. concern: renal impairment Acute renal failure 1. Definition: sudden, severe loss of function of kidneys, usually reversible 2. Etiologies: a. dehydration b. underlying conditions c. burns d. obstruction e. infection 3. Pathophysiology a. severe reduction in glomerular rate b. an elevated BUN c. decreased tubular reabsorption of sodium from the proximal tubule d. increase in sodium in distal tubules stimulated renin mechanism e. decrease renal blood flow f. cortical and tubular necrosis 4. Findings a. usually related to imbalances in fluids and electrolytes b. often nonspecific (edema, hypertension, decreased feeding, anorexia, lethargy, pallor, seizures) c. oliguria 5. Diagnostics a. history b. I & O c. laboratory testing d. radiographic (ultrasound, renal scan) 6. Management a. treatment directed at: 1. correcting underlying cause 2. managing complications 3. supportive therapy

I.

acute: fluids, electrolytes, dialysis (peritoneal, hemodialysis), antihypertensives, diuretics 7. Concerns: fluid status, acute and chronic renal damage, infection, cardiac failure Chronic renal failure (CRF) 1. Definition: progressive deterioration of kidneys so that they can no longer maintain normal chemical structure of body fluids under normal conditions 2. Etiology: immunological injury, congenital anomalies, underlying disease/condition 3. Pathophysiology: irreversible and permanent a. progressive nephron destruction b. distribution throughout nephron - uremia (urea in blood) c. final stage: end-stage renal disease (ESRD) is irreversible 4. Findings a. failure to thrive, anorexia, nausea and vomiting b. fluid and electrolyte imbalances c. hypertension, uremia, anemia, bleeding tendencies d. increased fatigue on exertion e. skin: sallow, muddy appearance 5. Diagnostics: history, physical exam, laboratory testing, radiographic studies 6. Management a. dialysis b. kidney transplant c. diet: modified (decreased sodium, potassium, phosphorus, protein; fluid restriction, high calcium) d. medications: antihypertensives, diuretics, erythropoietin, growth hormone, immunosuppressants (post transplant) e. monitor status (for example, laboratory reports) f. give psychological support 7. Concerns: bone demineralization, anemia, failure to thrive, retention of wastes; transplant concerns: kidney rejection and immunosuppression

b.

Genitalia Problems J. Cryptorchidism 1. Definition: failure of one or both testes to descend normally through inguinal canal into scrotum 2. Etiology/pathophysiology a. abnormal testes, or b. decrease in the hormonal stimulation necessary for descent 3. Management: wait up to one year for descent, medications (HCG) to assist in descent, or surgery (orchiopexy). Hypospadius 1. Definition: urethral meatus below normal placement on glans penis or anywhere along ventral (underside) surface of penile shaft 2. Etiology: idiopathic - related to genetics, environment, hormonal 3. Pathophysiology: incomplete development in utero 4. Management: surgical correction (with urinary catheter and stents post-op), post-op pressure dressing must not be removed by anyone other than the health care provider 5. Concerns: stenosis, chordee, body image/self esteem

K.

L.

Clubfoot (illustration ) 1. Etiology: congenital malformation of one or both feet 2. Findings: plantar-flexed foot/feet, with inverted heel and adducted forefoot. 3. Management: serial manipulation and casting after birth; possible surgery M. Genu varum (bowleg) and genus valgum (knock knee) 1. Etiology: congenital 2. Findings a. bowlegs (genu varum) common in infants and toddlers b. knock knees common in preschool age and older. 3. Management: most resolve spontaneously - pathologic forms may require night splints, manual manipulation, casting or surgery. N. Hip dysplasia 1. Definition: developmental dysplasia of the hips (DDH), or dislocation 2. Etiology: congenital, pre- and post-natal positioning

3.
4.

Pathophysiology: head of femur is improperly seated in acetabulum in hip (illustration ) Clinical manifestation a. limited abduction b. short femur on affected side (Galeazzi's sign) c. asymmetry of gluteal skin folds, d. waddling gait (bilateral dislocations) e. for children already walking, increased laxity Diagnostics a. physical exam/screening at birth b. check for Ortolani maneuver and Barlow's test c. radiographic studies Management: Pavlik harness, spica cast, traction, surgery, Bryant's traction Concerns: compliance, skin integrity, avascular necrosis from improper positioning of harness

5.

6. 7.

O.

P.

Q.

Scoliosis (illustration ) 1. Definition: lateral curvature of the of spine and rotation of vertebral bones 2. Etiology a. idiopathic b. associated with neuromuscular disorders or trauma (paralytic) c. congenital d. most commonly diagnosed during adolescent growth spurt 3. Pathophysiology a. dependent on type (idiopathic, congenital, paralytic) b. curved spine deforms rib, body develops compensatory curve to maintain posture and balance. 4. Findings a. visible curve (either C or S shaped curves) b. "rib hump," or asymmetric rib cage c. legs are different lengths d. waist angles uneven 5. Diagnostics: screening, radiographic studies 6. Management: a. initially, exercise and bracing to hold curve (Milwaukee brace - rarely used, Boston brace - plastic shell, or TLSO - custom-molded jacket) b. if curve progresses, surgery 7. Concerns a. body image/self esteem, pain and discomfort b. compliance with exercises and bracing; skin integrity, airway clearance Osgood-Schlatter disease 1. Etiology: idiopathic - possibly due to repetitive stress from sports related activity plus overuse of immature muscles and tendons. Usually self-limiting. 2. Findings a. knee pain/tenderness b. edema of tibial tubercle c. exacerbated by running and jumping 3. Diagnostics: physical exam, history, radiographic studies 4. Management a. avoid certain activities b. use elastic bandage on affected knee c. rest, ice, NSAID's Legg-Calve-Perthes disease: osteochondritis 1. Definition: aseptic necrosis of femoral head 2. Etiology: unknown - possible growth disorder 3. Pathophysiology a. disturbance in blood supply

b.
4.

ischemic aseptic necrosis of femoral head, usually self-limiting (illustration )

Findings a. insidious onset b. intermittent painful limp on one side c. increased pain after extended period of activity

5. 6.

7.

d. decreased range of motion Diagnostics: history, radiographic studies, bone scan, MRI Management a. bed rest b. non weight-bearing range of motion c. containment devices such as braces, casts, traction d. possible surgery Concerns: permanent deformity, decreased activity, skin integrity

Disorders of Red Blood Cells

E.

Sickle cell anemia (SCA) (illustration 1 illustration 2 ) 1. Etiology: autosomal recessive disease; 1 in 12 African-Americans carries the trait, while 1 in 375 is affected with sickle cell disease 2. Pathophysiology a. trigger - RBC's shape becomes sickled and clump b. generalized microvascular occlusion (vaso-occlusive crisis) 3. Findings a. result from i. obstruction caused by sickled RBCs ii. RBC destruction b. hypoxia c. organ dysfunction (spleen, liver, kidney) due to ischemia and infarction d. painful episodes 4. Therapeutic management a. aims of therapy i. to prevent sickling phenomenon ii. to trust medical emergency sickle cell crisis b. hydration c. oxygen d. analgesics e. antibiotics f. folic acid g. prophylaxis with penicillin recommended 5. Concern: sickle cell crisis 6. B-Thalassemia a. Definition: inherited blood disorder characterized by deficiencies in rate of production of specific globin chains in hemoglobin b. Etiology: autosomal recessive disorder, also known as Cooley's anemia c. Pathophysiology: abnormal, chronic production and destruction of RBC's resulting in insufficient amounts of normal circulating hemoglobin d. Findings i. severe anemia, pallor ii. microcytic RBC's iii. impaired growth iv. splenomegaly e. Management i. chronic transfusion therapy to maintain hemoglobin of ten gm/dl ii. referral for chelation counseling

7.

8.

Idiopathic thrombocytopenic purpura (ITP) (illustration ) a. Etiology: unknown, but often occurs one to three weeks after a febrile, viral illness b. Pathophysiology i. auto-immune disorder ii. platelets are killed and fewer are made iii. may be acute and self-limiting; or chronic c. Findings: excessive bruising, petechiae, internal bleeding d. Therapeutic management: steroids, immunoglobulins (IVIG), anti-D antibody, splenectomy for chronic disease Von Willebrand's disease a. Etiology: congenital (autosomal dominant) bleeding disorder; affects both males and females b. Pathophysiology

c.
d.

i. body makes too little von Willebrand's factor and factor VIII ii. prolonged bleeding time Findings: excessive bleeding of mucous membranes: gums, epistaxis (nosebleed), menorrhagia Therapeutic management: replacement of missing clotting factor, and/or administration of antidiuretic: desmopressin acetate (DDAVP

9.

Aplastic anemia (illustration ) a. Etiologies i. congenital (example: Faconi's anemia) ii. acquired due to exposure to overwhelming infection (hepatitis, HPV) iii. causative agents such as antineoplastic agents, chemicals, chloramphenicol b. Pathophysiology i. bone marrow stops making erythrocytes, leukocytes, and platelets. ii. result: pancytopenia c. Findings: petechiae, bruising, pallor, fatigue, myelosuppression d. Therapeutic management i. antithymocyte globulin (ATG), antilymphocyte globulin (ALG) ii. if severe, bone marrow transplant is treatment of choice a. b. c. d. ) Etiology: x-linked recessive disorder Pathophysiology: missing or defective factor VIII or factor IX blood components necessary for blood coagulation Findings: i. mild-severe prolonged bleeding; most often in muscles and joints (hemathrosis) ii. longterm loss of range of motion of affected joints Therapeutic management i. replacement of missing clotting factor, factor VIII concentrate ii. desmopressin acetate, a diuretic (DDAVP) iii. prophylactic treatment with clotting factor before surgery and some other procedures iv. ice, rest, elevation, immobilization, pressure to bleeding site

10. Hemophilia (illustration

11. Disseminated intravascular coagulation (DIC) (illustration

) Etiology i. secondary disorder of coagulation that complicates other disorders ii. triggered by endothelial damage such as trauma, shock, infections, hypoxia, liver disease b. Pathophysiology i. the first stage of the coagulation process is abnormally stimulated ii. clotting mechanism is triggered in circulation, thrombin is generated in greater amounts than the body can neutralize iii. rapid conversion of fibrinogen to fibrin with aggregation and destruction of platelets iv. local and widespread fibrin deposition in blood vessels causes obstruction and necrosis v. fibrinolytic mechanism causes extensive destruction of clotting factors c. Findings i. bleeding, bruising, petechiae ii. altered serum levels of clotting-related factors (increased PT, PTT, TT, decreased platelets, degraded fibrinogen) iii. clotting, hypoxemia, intracranial hemorrhage, progressive organ failure d. Therapeutic management i. control of underlying etiology ii. factor replacement iii. platelets, fresh frozen plasma (FFP) and RBC transfusions iv. vitamin K v. oxygen 12. Cardinal findings of cancer in children a. Unusual mass b. Pallor c. Sudden tendency to bruise d. Rapid, unexplained weight loss e. Change in vision or eye

f. g. h. a. b.

Recurrent fever Persistent headache, often with vomiting Change in balance or gait illustration 2 ) Definition: cancer of blood-forming tissues Pathophysiology of acute lymphoid leukemia (ALL) and acute myelogenous leukemia (AML) i. malignancies of the bone marrow and lymphatic system ii. unrestricted proliferation of immature WBCs iii. leukemia cells demonstrate the neoplastic properties of solid cancers iv. symptoms caused by infiltration and replacement of any tissue of the body with non-functional leukemia cells. v. highly vascular organs such as spleen and liver are most severely affected Findings i. acute or insidious onset, ii. anemia, thrombocytopenia, infection, bleeding

13. Leukemias - most common cancer in children (illustration 1

c.

d.

iii. lymphadenopathy, hepatosplenomegaly, bone or joint pain Therapeutic management i. combination chemotherapy ii. radiation if CNS involved iii. bone marrow transplant
illustration 2 )

Lymphomas (illustration 1 C.

Hodgkin's disease 1. Definition: malignancy of the lymph nodes 2. Pathophysiology a. neoplasm of lymphatic system b. characterized by giant, multinucleated cells (Reed-Sternberg cells) (illustration 3. ) Findings a. characterized by painless enlargement of lymph nodes, particularly in supraclavicular area b. anorexia, weight loss, malaise; painless; night sweats c. fever Diagnostics: lymph node biopsy Management a. chemotherapy and radiation b. treatment based on staging of disease (stages I-IV)

4. 5.

D.

E.

F.

Non-Hodgkin's lymphoma (illustration ) 1. Pathophysiology: a. proliferation of either B- or T- lymphocytes b. staging I-IV 2. Findings a. painless, enlarged lymph nodes in cervical or axillary region b. bone marrow and/or mediastinal involvement 3. Therapeutic management: combination of chemotherapy and radiation Brain tumors: medulloblastoma, astrocytoma, ependymoma, glioblastoma 1. Pathophysiology a. solid tumors of the nervous system b. most common solid tumor in children 2. Findings a. headache, vomiting, increased intracranial pressure, ataxia b. visual changes, seizures, bulging fontanel in infants, behavioral changes 3. Therapeutic management a. surgical debulking b. radiation c. chemotherapy Neuroblastoma 1. Pathophysiology

a.

b.

2. 3.

c. Findings a. firm, nontender mass in the abdomen that crosses the midline b. distant metastasis may cause periorbital edema (swelling around eyes) Therapeutic management a. treatment is dependent on staging criteria b. surgical debulking, chemotherapy, radiation

solid tumor found only in children under four years old primary sites are adrenal gland or retroperitoneal because it starts from embryonic neural crest cells of the adrenal gland and sympathetic nervous system. often has metastasized by the time it is diagnosed

Other Tumors Summary Overview of Other Tumors in Children:

G.

H.

Osteosarcoma (illustration ) 1. Pathophysiology a. originates from bone-forming mesenchyme b. creating malignant osteoid tissue c. most common in distal femur d. metastasis to lung 2. Findings: localized pain, limp, decrease in physical activity 3. Therapeutic management: surgical approach a. limb salvage with prosthetic bone replacement; or amputation b. plus combination chemotherapy before and/or after surgery Wilm's tumor 1. Pathophysiology a. solid tumor of the renal system b. possibly genetic in some cases c. most often affecting the left kidney d. tumor encapsulated for extended period e. is staged I-V at time of resection 2. Findings

I.

a. abdominal mass b. characteristically firm, non-tender c. confined to one side 3. Therapeutic management a. surgical resection b. combination chemotherapy, irradiation c. highest survival rate of all pediatric malignancies Retinoblastoma 1. Pathophysiology: congenital malignant tumor of the retina, with evidence of genetic inheritance in certain cases 2. Findings: cat's eye reflex (whitish glow in pupil); strabismus; red, painful eye 3. Therapeutic management a. radiation therapy, enucleation, chemotherapy b. treatment depends on stage of tumor, stages I-V

Viral Infections

Viruses are parasites that cannot reproduce or meet own metabolic needs. Skin cells react to virus with swelling, "vesiculation," or proliferation, sometimes warts. Most viruses are associated with rashes (characteristic of each disorder, such as chicken pox, rubella, roseola)

Pediatric Neurology

Abnormal posturing is an ominous sign A positive Babinski is normal in children until one year of age Myelinization continues until adolescence Abnormal CSF findings include: decreased glucose, positive culture, and cloudy appearance Due to pharmacokinetics and dynamics, common side effect of the majority of anti-convulsants include drowsiness, ataxia, lethargy, anorexia, nausea. Sometimes dyscrasias or liver damage can occur; hence, these children need periodic tests of blood and of liver enzymes. Febrile seizures are generally a one-time event, though there may be a familial predisposition. Children are more likely than adults to have neuromuscular or extrapryamidal side effects from psychotherapeutic drugs. Clinical effectiveness of anticonvulsants varies with the drug's serum level, mechanism of action, pharmacokinetics and dynamics. The effects also may vary from child to child. A newborn's brain is about two-thirds the size of an adult's, and reaches 80% adult size in one year. The sudden appearance of a fixed or dilated pupil is an emergency. The progression from decorticate posture to decerebrate posturing, and then to flaccid paralysis, indicates deterioration of neurologic function. Do not do any diagnostic tests that require head movement until cervical spine injury has been ruled out. Children with congenital neurological disabilities will often develop complications in other body systems. Cerebral palsy is a neuromuscular disorder. It may bring with it certain problems in perception, language, and/or intellectual function. Acute bacterial meningitis is a medical emergency, requiring swift action and treatment. The care of the unconscious child focuses on respiratory management, neurological assessment, monitoring intake and output, providing appropriate medications and evaluating outcomes. The primary indicator of neurological status is LOC (level of consciousness). Status epilepticus is an emergent situation. Do not restrain a child experiencing a tonic-clonic seizure, and never place anything in his mouth. In head trauma, the primary mechanism of injury is acceleration-deceleration accidents. Bleeding from the nose or ears calls for evaluation.

Pediatric Cardiovascular

In a cardiac history, include poor weight gain, chronic respiratory infection, activity intolerance, and fatigue during eating.

Oxygen is a drug that requires a prescription and frequent monitoring. Cardiac catheterization serves many purposes: diagnostic, interventional and electrophysiologic. It also monitors cardiac oxygen saturation, pressure changes and anatomic defects. CHF signs usually show either left or right sided heart disorders. These signs may include increased heart rate, adventitious lung sounds, cyanosis, edema, hepatosplenomegaly, and distended neck veins. Acquired cardiac disorders include bacterial endocarditis, acute rheumatic fever, hyperlipidemia, Kawasaki disease, and cardiomyopathy. Electrodes for cardiac monitoring are usually color coded: white (upper right), black (upper left), green (lower right), and red (lower left).

In cyanotic heart disorders, major concerns are polycythemia or increased hemoglobin and hematocrit. These can lead to thrombus.

Pediatric Respiratory

The principal functions of the respiratory tract are to allow air movement (ventilation) and exchange (diffusion) of oxygen and carbon dioxide. Children's airways are smaller, more flexible and shorter than adult's and are therefore more prone to obstruction than adults. Stridor usually indicates an upper airway concern, while wheezing indicates a lower airway disorder. Conditions that increase or decrease compliance and/or resistance will make breathing harder. Signs of increased breathing work are tachypnea, retractions, abnormal positioning, shortness of breath and fatigue. Respiratory rate is an important indicator of respiratory status. Central cyanosis in a newborn usually means severe hypoxia and possible cardiac etiology. Acrocyanosis is a common finding in a newborn. Asthma is not a disease but an inflammatory disorder. Asthma is not wheezy bronchitis. The incidence and severity of respiratory tract infections and disorders is related to the child's age, size, natural defenses, underlying disorder and agent involved. After a tonsillectomy child may bleed for up to several weeks. Epiglottitis, acute tracheitis and status asthmaticus are acute medical emergencies. The best way to stop the spread of RSV is meticulous hand washing. RSV is transmitted by direct contact with the fomite.

Pediatric Endocrine

The body secretes hormones at various times during the day (influences of diurnal and circadian rhythm). Normal hormone levels are related to age and stage of puberty. The pituitary gland stimulates target organs to produce specific hormones; when sufficient, these in return signal pituitary to stop stimulation (negative feedback loop). Untreated infant hypothyroidism will lead to mental retardation. Associated terms for hypopituitary function include: short stature, constitutional delay, dwarfism. A major concern of precocious puberty is rapid bone growth, which can result in early fusion and short stature. Children with SIADH develop an expanded circulatory volume but not edema. Because oral potassium tastes very bitter, mix it with a little strongly flavored fruit juice. For a child with an endocrine disorder, never discontinue medication abruptly.

The vast majority of children with new-onset diabetes mellitus type 1 (IDDM) will experience a "honeymoon" period when their bodies secrete insulin and their need for exogenous insulin decreases. Blood glucose monitoring by finger-stick reflects glucose currently and for last several hours; glycosylated hemoglobin levels indicate long-term compliance and true diabetic status. Never freeze, heat or vigorously shake insulin. When insulin is absent, the body cannot properly metabolize fats, proteins and carbohydrates. The focus of diabetic management is the inter-relationship of diet, activity and insulin administration.

Pediatric Gastrointestinal

Infants and children have a much smaller stomach capacity than adults. Peristaltic waves may reverse occasionally during infancy; gastric esophageal reflux is very common in infants. Secretory cells don't reach adult levels until two to three years of age. The GI tract has both intake (fluid, minerals, vitamins, etc.) and output functions. Whenever a newborn coughs, chokes and turns blue with feeding, suspect tracheoesophageal fistula. Any newborn failing to pass meconium stool within the first 24 hours of life and who is prone to constipation or decreased frequency of stooling in the first month of life, should be evaluated for Hirschsprung's Disease. The treatment of metabolic acid-base disturbance is oriented toward correcting the underlying problem. Dehydration can lead to shock. Dehydrated infants and children face greater morbidity risk than adults because children differ in body composition and metabolic rate, and their fluid-regulation systems have not matured. Potassium should only be added to IV fluids when the urine output is sufficient. One Gm of diaper weight = one cc of urine. When assessing diarrhea or constipation, remember the acronym ACCT: amount, color, consistency, and time (duration). Bilious vomiting indicates source below the ampulla of Vater.

Pediatric Genitourinary

The kidney's function is to maintain, in equilibrium, the composition and volume of body fluids. Kidney function in an infant is nearly that of an adult by 12 months of age. Children with urine output less than one ml/Kg/hour should be closely monitored for possible renal failure. Acute renal failure should be suspected in a child with decreased urine output, edema and/or lethargy, and who is dehydrated, recovering from surgery or in shock. In managing HUS, the goals are to control hematologic manifestations and any renal complications. UTI management aims to eliminate the underlying cause, detect and correct abnormalities, and prevent recurrences. The effects of hypokalemia or hyperkalemia can be devastating. UTIs are extremely common in young children, girls more than boys. In a child with ambiguous genitalia, the criterion for choice of gender and rearing is not genetic sex, but the infant's anatomy.

Pediatric Musculoskeletal

Since many musculoskeletal disorders begin with trauma, it is important to assess ABC (airway, breathing and circulation) first. Open fractures increase the risk of infection. Immobilization has multi-system effects. For a child with a fracture, it is important to assess the five P's of ischemia: 1. Pain and point of tenderness

2. 3. 4.

Pulse - distal to the facture Pallor Paresthesia

5. Paralysis Children with structural defects/disorders require regular follow-up evaluation until they reach skeletal maturity. Children in casts or traction need to be monitored for alterations in skin integrity routinely. Children under one year of age generally do not experience fractures. Because children's soft tissues are so resilient, dislocation and sprains are less common.

Pediatric Temperature-Related

The extent of a burn injury is expressed as percentage of total body surface area (TBSA) The larger the percentage of TBSA that is burned, the greater the risk for burn shock. In managing alterations in skin integrity, it is necessary to individualize the type of treatment and medications to the particular causative agent. If you wouldn't put it into an eye, don't put it into a wound. Wounds heal by the process of moist wound healing and occlusion. Dry wounds do not heal. Wound debridement promotes healing and prevents infection. Immediate care for a major burn is ABC: airway establishment and patency, breathing and absence of respiratory distress, and circulation with fluid initiation. Potassium should not be administered during the initial oliguric phase of a burn injury, but should be added when diuresis occurs.

Pediatric Hematology

For a child with altered platelet function or bleeding disorder, do not administer acetylsalicylic acid (aspirin, ASA) or take rectal temperatures. Perform invasive procedures very cautiously. Children with low WBC may not exhibit common findings of infection such as purulent drainage. In a febrile client with granulocytopenia, give antibiotics immediately because this child risks rapid, overwhelming sepsis. Morphine is the medication (or opioid) of choice for pain in children with sickle cell disease.

Pediatric Oncology

Findings of pediatric malignancies vary according to the child's age, location and type of tumor, and extent of disease Cure rate is improving for most types of pediatric malignancies; however the late effects of treatment are of increasing concern and incidence. Children typically have longer treatment plans than adults due to their increased metabolic rate and rate of cell turnover. Leukemia affects not only the blood, but can metastasize to major organ systems (extramedullary disease), including the central nervous system. Nursing care includes monitoring the child for the development of acute complications of treatment including fever, bleeding, and anemia. Pediatric oncologic emergencies include: acute tumor lysis syndrome, superior vena cava syndrome, septic shock.

anasarca anergy apocrine glands Arnold-Chiari deformity automaticity azotemia bactericidal bacteriostatic chordee chronotropic compartment syndrome

craniosynostosis crepitation cretinism decerebrate posturing decorticate posture eccrine sweat glands eschar euthyroid graft-versus-host reaction (GVHR) hemostasis Hering reflex histamine involucre jaundice McBurney's point Meckel diverticulum meconium melena microcephaly mucokinesis mucositis neuroleptic malignant syndrome (NMS) neutropenia oncogenes perfusion phimosis polydactyly pulse oximeter reticulocyte sequestrum surfactant ultrafiltration urethritis vesicant Pediatrics

ABO blood types Acetabulum of right hip bone Acute lymphcytic leukemia Anal canal Aplastic anemia Atopic dermatitis Blood composition Bone tissue Brain Bullae of impetego Burns Cascade theory of coagulation Cerebrum Cerebrospinal fluid Chronic lymphocytic lukemia Conduction system of the heart Connective tissues Contact dermatitis Cranial nerves and their distribution Diaper rash Digestive system Endochondral ossification

Fontanels of infant skull Granulocytopoiesis Heart Herpes labialis Herpes simplex Idiopathic thrombocytopenic purpura Larynx Left foot Louse Lumbar puncture Lungs Lymph node Lymphatic system Nerve cell from cerebral cortex Paranasal sinusitis Pediculosis capitisis Peripheral cutaneous nerves (anterior view) Peripheral cutaneous nerves (posterior view) pH scale Projection of heart Reed-Sternberg cells Respiratory system Right femur, front view Scabies Scoliosis Simian and normal palmar creases Skeletal muscle Sickle cell anemia (a) Sickle cell anemia (b) Skin section Spinal cord Stomach Synapse Two views of nasal cavity Three mast cells in bone marrow Thyroid glands and related structures Tinea capitis Tinea corporis Tinea versicolor Types of blood cells Types of casts Types of fractures and terminology Urinary system Ventricles of the brain Vermiform appendix Vili of small intestine Wound healing