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Pediatric Nursing
I. Pediatric Cardiovascular

Congenital Heart Defects A. General concepts 1. Definition: present at birth - consequences are congestive heart failure and hypoxemia 2. 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 3. 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 g. cyanosis and/or pallor may be associated with poor perfusion h. unusual pulsations 4. Physical consequences of congenital heart defects a. increased workload; pulmonary hypertension; decreased systemic output; cyanotic defects

b. can lead to hypoxemia and polycythemia
c. concern: formation of thrombus with embolus d. blood flow patterns may be affected B. Acyanotic defect - infant/child is "pink" but child may develop cyanosis 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. Common types:

C. Cyanotic defect: infant/child is usually "blue", but child may appear pink 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. Diagnostics 1. EKG: noninvasive, painless, infants and young children may require mild sedation 2. Cardiac catheterization a. presence of diaper rash may postpone procedure b. preparation depends on level of growth and development c. post procedure – child may have difficulty complying with keeping insertion point in correct position E. 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 e. improve cardiac function f. remove accumulated fluid g. decrease cardiac demands h. improve tissue oxygenation 4. Nursing interventions a. recognize CHF FINDINGS OF CONGESTIVE HEART FAILURE

b. monitor height, weight, vital signs, pulses, pulse oximeter, intake and output c. medications: digoxin (Lanoxin), furosemide (Lasix) or chlorothiazide (Diuril), ACE inhibitors: Capoten, Vasotec d. monitor serum potassium level e. recognize and treat pain appropriately: pharmacological and nonpharmacological interventions f. maintain a safe environment g. conserve energy h. maintain proper nutrition, with small, frequent feedings – monitor sodium intake, fluid restriction may be required i. support and discuss treatment with parents j. place in proper position - slanting position with head elevated, older babies in infant seats, occasional knee-chest 5. Teaching points a. inform parents about findings of infection b. discuss possible behavior changes the child may exhibit

c. counsel parents about high-risk children requiring antibiotic prophylaxis for dental work Acquired Cardiovascular Disorders occur after birth as a result of infection, autoimmune responses, environmental or familial tendencies F. 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) 5. Nursing interventions a. encourage screening for at risk children with family history b. teach dietary guidelines or refer to dietician G. Bacterial endocarditis: inflammation of the valves of the inner lining of the heart 1. Etiology: possible causative agents a. strept b. staph c. fungi 2. Pathophysiology a. organisms enter blood stream (vascular dissemination) b. form vegetation on endocardium 3. Diagnostics a. EKG changes b. sedimentation rate evaluated c. WBC elevated d. blood cultures 4. Management a. high doses of antibiotics 5. Nursing interventions a. monitor for signs of infection b. provide rest periods 6. Teaching points a. need for long-term IV therapy b. side effects of antibiotics c. high-risk children need prophylactic antibiotic therapy before dental work or other invasive procedures Pediatric Respiratory Respiratory Infections A. General concepts 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

II.

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 5. Nursing interventions a. chest physiotherapy b. provide nutrition and encourage fluids - Jello, soup, puddings c. promote rest and comfort d. prevent spread of infection e. ease respiratory efforts - warm mist 6. Teaching points a. handwashing b. avoid contact with affected children B. 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 C. 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 also 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. Definition: related to development delay in lung maturation 2. Etiology a. premature infants: usually due to surfactant deficiency b. newborns: birth asphyxia, multiple gestations, diabetic mother c. older children: trauma, drowning 3. 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 c. increased alveolar surface tension d. impaired gas exchange

e. increased pulmonary resistance f. hypoperfusion

4. Findings

Findings of Respiratory Distress Syndrome The transition from respiratory-related to systemic related indicates a progressive severity of the disease 1. Respiratory related a. rales b. expiratory grunting c. nasal flaring d. retractions e. tachypnea f. apnea 2. Systemic related a. flaccid body b. nonresponsive c. hypotension d. shock

a. tachypnea, increased respiratory effort b. paradoxic "seesaw" respirations c. nasal flaring d. substernal retractions e. expiratory grunt, possible apnea f. cyanosis g. hypoxia 5. Diagnostics a. physical exam, pulse oximeter b. serum: ABG's, glucose, calcium c. chest radiograph confirmation – diffuse pattern over both lung fields that resembles ground glass 6. Management: a. oxygen therapy (possible mechanical ventilation) and chest physiotherapy to maintain ventilation and oxygenation b. medications: possible medications: surfactant, prophylactic antibiotics, diuretics, inotropes, methyl-xanthines 7. Nursing interventions: a. frequent respiratory assessment. b. maintain acid base balance and tissue perfusion c. electrolyte status d. suction as indicated 8. Teaching points: a. inform parents about equipment b. positioning of infant c. need for meticulous care by nurses due to subtle changes possible in infant’s oxygenation status E. 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 d. provide supplemental oxygen at home as needed 5. Nursing interventions: a. provide rest periods b. observe for fluid overload or pulmonary edema c. provide age appropriate toys d. frequent respiratory assessment F. Apnea: cessation of breathing for over 20 seconds 1. Etiologies a. prematurity b. foreign-body aspiration, drowning, or trauma

2.

3. 4. 5.

c. incorrect positioning d. gastroesophageal reflux e. infections f. seizure g. hypoglycemia 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 Findings a. depend on type (above) b. color changes, hypotonia Diagnostics: laboratory tests, chest x-rays, EEG, ECG, pneumocardiography, upper GI series Management a. home apnea monitoring and basic life support (BLS) training to family b. medications: based on type and underlying condition

Respiratory Conditions G. Upper respiratory tract infections (URI)

1. Etiology: often acute viral nasopharyngitis, or the "common cold" 2. Pathophysiology a. organism invades mucous membranes b. edema, vasodilation and increased mucus production c. usually self-limiting

3. Findings: nasal congestion, sneezing, colored nasal discharge, low grade fever, cough, irritability 4. Management: a. medications: antipyretics, decongestants (oral or nasal), analgesics b. cool mist humidifier c. adequate fluids d. rest e. assess for presence of earache (otitis media), temperature over 38.3 degrees Celsius and refusal to eat - may indicate complications H. Sinusitis

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 a. medications 1. antibiotics 2. antipyretics 3. analgesics 4. decongestants 5. antihistamines 5. Nursing care a. monitor respirations b. warm mist (possible)

I.

Acute pharyngitis 1. Etiology: streptococcus (bacterial); or viral (often accompanies tonsillitis) 2. Findings a. bacterial i. "sicker" with sudden onset ii. very sore throat iii. high fever iv. headache b. viral i. gradual onset ii. fever iii. cough, rhinitis 3. Diagnostics a. throat culture b. rapid strep 4. Management a. medications: penicillin for strep, possible antipyretics and analgesics 5. Nursing interventions a. monitor for complications such as acute rheumatic fever and acute glomerulonephritis b. monitor fluid intake c. moisture compress to neck – cold or warm d. cool liquids or ice chips 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 a. palatine tonsils usually visible during oral exam b. pharyngeal tonsils are also known as the adenoids 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 done after 3 episodes of tonsillitis in one year c. adenoidectomy may be done with tonsillectomy, if adenoiditis is present. 6. Nursing interventions a. assess for frequent swallowing – bleeding may be the cause b. assess any vomitus c. place child on side d. medicate for pain as needed e. avoid fluids with red or brown color f. soft foods – avoid highly seasoned food 7. Teaching points: teach parents a. child needs quiet activity b. monitor for bleeding c. antipyretics and analgesics – no ASA d. soft foods as throat is very sore K. Croup syndromes (including laryngitis, tracheitis, epiglottitis)

Similarities and Differences of Diseases Included in the Croup Syndromes

1. Definition: several airway-blocking infections, common in children

a. signs of croup i. inspiratory stridor ii. harsh/brassy cough, barking cough iii. hoarse voice

iv. respiratory distress b. types, by primary area affected: i. subglottal area: acute spasmodic croup, laryngitis, laryngotracheobronchitis (LTB), tracheitis ii. supraglottal area: epiglottitis

2. Etiology a. usually viral b. occasionally bacterial (tracheitis, epiglottitis) c. younger children with "true croup" (spasmodic croup) d. older children with tracheitis and epiglottitis 3. Pathophysiology: mucosa inflamed; edema narrows the airway 4. 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" i. drooling ii. dysphagia (difficulty swallowing) iii. dysphonia (hoarse voice) iv. distressed inspiratory efforts 5. 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. epiglottitis is a medical emergency; tracheotomy may be necessary 6. Nursing interventions a. teach parent and child signs of impending airway obstruction b. report increased pulse, respirations, retractions, increased restlessness c. never attempt to directly visualize epiglottis with tongue depressor; it could precipitate laryngospasm

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. enters body via nose or eye c. leads to edema, mucus accumulation and cellular debris which obstruct bronchioles d. can progress to atelectasis 3. Findings - infection is rare in children older than 2 years of age a. usually mild URI b. sneezing, productive cough, low-grade fever, rhinorrhea (nasal discharge), adventitious lung sounds c. otitis media and conjunctivitis may also be present 4. Diagnostics: history, RSV/viral nasal washing, chest x-ray 5. Management depends on severity a. mild: fluids, humidification, rest b. severe: hospitalization, antiviral, IV fluids, possibly bronchodilators, steroids and mechanical ventilation. Maintain contact secretion precautions c. prophylaxis: respiratory syncytial virus immune globulin in high risk infants 6. Nursing interventions a. admit each child to single room or with other RSV infected children b. teach handwashing and use of contact precautions c. assess fluid volume status 7. Teaching points a. check immunization schedule. RSV – IVIG may interfere with immune response. No vaccines for 9 months after child receives these M. Pneumonia: inflammation of lung parenchyma

1. Etiology: usually classified by anatomic distribution or pathogen a. most commonly viral b. sometimes bacterial c. sometimes aspiration of foreign substance 2. 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 d. may occur as a complication of another illness 3. Findings a. can be abrupt or insidious b. adventitious lung sounds, fever, malaise, nonproductive cough, restless, lethargic c. progressing to more severe with retractions, respiratory distress, productive cough 4. Diagnostics a. increased WBC - chest x-Ray reveals consolidation in lungs or presence of fluid 5. 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 risk d. administer pneumococcal vaccine to at risk individuals 6. Nursing interventions a. frequent assessment of respiratory status b. cool mist tent - change clothing frequently c. cluster nursing care N. Aspiration of foreign body 1. Etiology: child aspirates solids, liquids, vegetative matter into air passages, most common in older infants and children up to three years of age 2. Pathophysiology: most substances become lodged in bronchi, and severity is determined by location, substance aspirated and extent of obstruction 3. Findings: sudden coughing, gagging, wheezing, cyanosis, dyspnea, and stridor 4. Diagnostics: chest x-ray, fluoroscopy, bronchoscopy 5. Management: direct laryngoscopy or bronchoscopy to remove object, then supportive therapy 6. Nursing interventions a. recognize signs of FB aspiration b. administer back blows or Heimlich maneuver as indicated 7. Teaching points a. parents need to know most likely causes to prevent aspiration 1. peanut butter 2. balloons 3. aluminum tabs from soda cans 4. paper clips O. Long-term respiratory dysfunction: asthma/reactive airway disease 1. Definition: chronic inflammatory disorder of the airways, manifested by periods of exacerbations and remissions; has an allergic component 2. Etiology a. genetic predisposition b. triggers are: allergens, infection, stress, exercise, medical conditions, medications. c. types: intrinsic, extrinsic, occupational

3. Pathophysiology

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

c. later phase reaction (additional inflammation and hyperresponsiveness) d. bronchospasm and obstruction cause most symptoms 4. 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 5. Diagnostics a. physical exam, history b. pulmonary function tests (PFT) c. bronchodilator treatments, chest x-ray, skin testing, CBC with differential d. allergy testing 6. 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 1. long term control (preventor) medications: to achieve and maintain control of inflammation; also called controllers 1. corticosteroids - anti-inflammatory 2. cromolyn sodium - nonsteroidal anti-inflammatory 3. nedocromil - anti-allergic and anti-inflammatory 4. long acting beta adregenics (Albuterol) used for acute exacerbations 5. methylxanthines (theophylline) bronchodilator 6. leukotriene modifiers (Zileuton) - mediator of inflammation 2. quick relief (rescue) medications to treat acute symptoms and exacerbations 1. short acting beta adrenergics 2. anticholinergics 3. systemic corticosteroids 7. Acute management a. use of bronchodilators b. steroids (inhaled, IV and/or oral) c. oxygen, IV fluids, possibility of intubation 8. Nursing Interventions a. monitor child for respiratory distress and/or need for nebulizer treatments. b. place semi-high Fowler’s position c. stay with child if at all possible - have parent stay during acute phase of illness d. monitor fluid volume status 9. Teaching points a. child and family must comply with medications and treatments b. correct use of metered-dose inhaler (MDI) with a spacer c. risks include overuse of bronchodilators 10. Complications a. exercise-induced bronchospasm – acute and reversible, usually stop in 20-30 minutes b. status asthmaticus – emergency situation P. Cystic fibrosis 1. Definition: inherited autosomal recessive trait - abnormal mucus secretion and obstruction 2. Etiology a. genetic origin b. basic defect exocrine gland dysfunction 3. Pathophysiology a. mucous glands produce a thick mucoprotein that accumulates instead of a thin freely flowing secretion

b. affects respiratory, gastrointestinal and reproductive systems 4. Findings a. gastrointestinal 1. meconium ileus 2. steatorrhea 3. prolapse of rectum 4. failure to grow b. respiratory 1. thick tenacious mucus causes patchy atelectasis cough 2. barrel-shaped chest 3. clubbing of fingers and toes 5. Diagnostics a. sweat chloride test - iontophoresis - Pilocarpine over 60 mEq/L is diagnostic of CF b. chest x-ray: patchy atelectasis c. stool analysis 6. Management a. CPT b. medications: bronchodilators, antibiotics (Pseudomonas aeruginosa - Cepacia, and staph aureus) c. D-Nase - decreases viscosity of mucus d. enzymes administered with meals and snacks to replace pancreatic enzymes 7. Nursing interventions a. high protein, high caloric well balanced diet, vitamins ADEK should be added b. frequent hospitalizations to treat respiratory infections c. monitor IV fluids III. Pediatric Neurology III. Pediatric Neurology A. General concepts: 1. Assessment of cerebral function: 2. Children under 2 unable to respond to directions 3. Infants primarily reflexive 4. Observe spontaneous and elicited reflex responses – having parent present may be helpful 5. Include family history and health history 6. Know stage of growth and development and developmental milestones Increased intracranial pressure (ICP)

B.

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Etiology a. congenital or acquired (from trauma, lesion, infection) b. also see Pediatric Oncology (See section XI of this lesson) Pathophysiology: swelling caused by irritation or bleeding into brain tissue Findings a. infant: bulging fontanels, widened cranial sutures, high-pitched crying, irritable

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b. child: headache, nausea, vomiting, lethargy, diplopia, seizures Diagnostics:

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a. CAT Scan – MRI Management a. principle of maintaining cerebral perfusion pressure (CPP), which equals mean arterial blood pressure minus ICP b. medications: osmotic diuretics, antihypertensives, anti-seizure, fentanyl, Versed and Vecuronium may be added c. maintain patient airway: mechanical ventilation, oxygen d. in severe cases, ICP monitor – ventricular tap may be required to relieve accumulated CSF e. minimize external stimuli 6. Nursing interventions: a. observe/monitor – LOC, pupillary reaction and vital signs/neuro signs b. pain management should be monitored c. nutrition – may require tube feeding if long term d. positioning – HOB is elevated 15-30 degrees head is maintained in midline. e. cluster care to allow periods of rest f. may not bathe child if ICP is unstable g. may need artificial tears h. support family Hydrocephalus - imbalance in the production and absorption of CSF in the ventricles 1. Etiology: congenital, acquired, or idiopathic 2. Pathophysiology: depends on type a. communicating: impaired absorption of CSF within the subarachoid space 5.

3.

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.

D.

infant (signs of increased intracranial pressure) I. bulging fontanels, increased head circumference II. "setting sun" eyes, pupils slow to constrict to light III. eats poorly IV. high-pitched cry V. variable pulse, changes in respirations c. older child (signs of increased intracranial pressure) I. headaches II. dizziness III. vomiting IV. diplopia V. ataxia 4. Diagnostics: a. CT SCAN – MRI I. sedation may be required 5. Management a. surgical placement of ventriculoperitoneal (VP) shunt in neonates, infants and older children placed on unoperated side to prevent pressure on operative site b. monitor for increased intracranial pressure c. measure head circumference d. no scalp vein IV lines 6. Nursing interventions: a. neuro and vital signs b. assess pain level – acetaminophen with or without codeine c. observe for abdominal distention for possible catheter complications d. NPO – feed after bowel sounds return e. monitor fluid volume status f. monitor head circumference g. keep diaper off perineal dressing 7. Teaching points a. teach family signs of infection I. elevated temperature II. poor feeding III. irritability IV. altered level of consciousness b. home care I. no contact sports II. child should be as active as possible. 8. Complications: shunt infection and malfunction Seizures 1. Definition: brief malfunctions of the brain's electrical system that result from cortical neuronal discharge 2. Etiology: cerebral, biochemical, post-traumatic, febrile, idiopathic, most common neurologic dysfunction in children 3. Pathophysiology a. trigger electrical discharges affecting nearby normal cells b. spreads throughout brain reaching midbrain reticular regions c. possible generalized seizure with neurological symptoms 4. 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)

b.

E.

may be nonrecurrent (acute) such as febrile episode or recurrent (chronic) such as epilepsy 5. Diagnostics – goal is to ascertain the type and cause of seizure. a. detailed and accurate history b. physical exam to include neurological assessment c. CBC – glucose level d. skull films, CT- MRI – EEG Epilepsy 1. Definition: chronic seizure disorder with recurrent and unprovoked seizures 2. Etiology: unknown. international classification: partial and generalized a. partial: limited to a particular local area of the brain I. most common I. simple partial with motor signs II. simple partial with sensory signs III. complex partial with psychomotor seizures b. generalized: involves both hemispheres of the brain I. most common I. tonic-clonic (grand mal) II. absence (petit mal) 3. Pathophysiology: see seizures 4. Diagnostics: toxicology screen, ABG’s, electrolytes, anticonvulsant drug level, possible lumbar puncture

g.

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6.

Management: see seizure precautions, seizure care, postictal care a. supportive: I. airway suction and oxygen II. safety – loosen clothing, place on floor, clear area, and remove restrictive clothing b. medications – antiepileptic drugs Nursing interventions a. observe seizure – protect from injury I. order of onset II. movement III. face IV. eyes V. respirations

F.

VI. incontinence of urine or stool VII. duration VIII. do not restrain b. assess postictal status c. stay with child until fully conscious 7. Teaching points a. long-term care medication regime b. child can participate in most activities for age c. assess trigger factors -possibly video games with flashing lights if sensitive Neural tube defects (NTDs) 1. Anencephaly - degeneration of the brain to a spongy form mass with no bony covering a. most serious b. both cerebral hemispheres are absent c. condition is incompatible with life beyond a few days 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 I. meningocele: encases meninges and spinal fluid but no neural elements; no neurological deficit II. meningomyelocele: contains meninges, spinal fluid, and nerves; varying neurological deficit findings vary widely to degree of spinal defect - the higher the location the more symptoms the infant will have. d. diagnostics: I. physical exam II. MRI – ultrasound – CT- myelography III. WBC e. nursing interventions I. initial care I. protect sac from rupturing and drying out II. assess for presence of latex allergy III. moist sterile nonadherent dressing –usually saline IV. fluids V. no diaper VI. positioning – on abdomen VII. ROM VIII. early surgical closure of lesion IX. avoid rectal temperatures II. long term I. protect skin integrity II. maintain bladder and bowel function III. support musculoskeletal integrity f. teaching points I. teach parents how to measure head circumference after closure II. may require VP shunt III. findings of infection Cerebral palsy (CP) 1. Etiology a. disorders characterized by early onset and impaired movement and posture; non-progressive - missed developmental milestones b. cause: abnormality in extrapyramidal or pyramidal motor system (cortex, basal ganglia, cerebellum) c. can occur prenatally, perinatally, or postnatally c.

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may be accompanied by perceptual problems, language deficits and intellectual involvement e. usually diagnosed by exclusion Pathophysiology a. classifications: athetoid, spastic, ataxic, mixed Classification of Cerebral Palsy

d.

1. Athetoid • Characterized by slow irregular twisting snakelike movements occurring in the upper extremities • Involuntary movement of oral muscles which may result in drooling and dysarthria 2. Spasticity • May involve either one or both sides • Impaired fine and gross motor skills • Individual's attempts at motion increase the abnormal posturing and overflow of motion to other parts of body • Hypertonic movements 3. Ataxic • Rapid repetitive movements performed poorly • Poor integration of movements when individual reaches 4. Mixed • Combination of athetosis and spaticity

H.

associated defects: mental retardation and cognitive impairment, hearing or visual losses, attention deficit disorder (ADD) c. high metabolic rate, leading to increased calorie expenditure 3. 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, MRI 5. Management a. establish locomotion, communication and self-help b. gain optimal appearance and function c. correct associated problems as effectively as possible d. promote socialization 6. Nursing interventions a. early recognition- assess children at risk b. treat what child demonstrates c. ROM d. teach ADL- dressing, feeding, walking e. incorporate play into treatment plan 7. Teaching points a. multi-disciplinary approach b. speech training is begun early c. long term problem and a financial concern d. offer support for family resources 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

b.

3.

mental retardation (varies from severe retardation to low average intelligence) d. dermatologic changes e. incomplete embryogenesis Findings a. facial features I. brachycephaly II. flat occiput III. protruding tongue IV. high-arch palate V. almond shaped eyes VI. low slung ears b. body features I. short, broad hands with simian crease

c.

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II. short, broad neck III. dry skin IV. large space between great toe and second toe Diagnostics a. physical exams b. chromosome analysis Management a. supportive: specific to body system affected b. promotion of developmental progress c. cognitive Nursing interventions a. treat what the child exhibits b. assess sight and hearing c. allow extra time when giving instructions d. support family Teaching points a. genetic counseling b. long-term disorder c. financial referrals

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

d. ascending flaccid paralysis 3. Findings a. preceded by mild influenza-like illness or sore throat b. gradual progression of symptoms i. acute - onset of findings ii. plateau – findings remain constant iii. recovery – begins to improve and progress to complete recovery, usually 2 to 3 weeks after onset. c. muscle tenderness, paresthesia usually ascend from feet/legs upward progressing to total paralysis 4. Diagnostics: a. physical assessment b. CSF analysis c. EMG findings – acute muscle denervation 5. Management a. short term: supportive and symptomatic b. based on physiologic needs and body system affected: possible tracheostomy, ventilatory support, IV fluids and plasmapheresis c. long-term: integumentary, musculoskeletal and respiratory systems d. medications: IV immunoglobulin and steroids, possible immunosuppressive agents 6. Nursing interventions: a. assess for progress of disease especially swallowing and breathing b. neuro and vital signs c. assess skin integrity d. may require tube feedings e. maintain Foley f. ROM g. support child and family 7. Teaching points

a. multi-disciplinary approach b. long-term process c. may have residual disability J. Botulism - acute flaccid paralysis 1. Etiology a. food poisoning from anaerobic bacillus: clostridium botulisum contaminated food b. three types: infantile, classic, wound 2. Findings a. CNS findings usually appear within 12 to 36 hours b. blurred vision, diplopia, lethargy, vomiting and dysphagia, weakness, difficulty speaking, life threatening progressive respiratory paralysis 3. Management a. supportive: dependent on body system affected b. protect ventilation, respiration, and provide nutrition c. medications i. botulism antitoxin 4. Nursing interventions a. observe for and report signs of neuromuscular weakness b. child will tire easily c. assess for swallowing difficulties 5. Teaching points a. child must rest during recovery b. normal bowel elimination may not return for a while; no enemas or cathartics

K. Reye's syndrome (toxic encephalopathy) 1. Etiology: follows a common viral illness, drugs (salicylate association), toxins, genetics 2. Pathophysiology a. acute noninflammatory encephalopathy and hepatopathy 3. Findings a. related to cerebral edema and fatty changes in liver b. viral upper respiratory infection c. fever d. severe nausea and vomiting

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e. profoundly impaired consciousness f. liver dysfunction g. coma Diagnostics: liver biopsy a. ammonia levels b. possible lumbar puncture Management a. supportive: dependent on body system affected b. avoid aspirin and other salicylates in children with viral diseases such as influenza or varicella disease Nursing interventions a. assess children at risk b. continuously monitor affected child c. neuro and vital signs d. assess IV lines, Foley e. may have N/G tube f. monitor fluid volume status Teaching points a. keep parents informed about status and equipment.

Traumatic Neurological Conditions L. Head trauma 1. Etiology: force to the scalp, skull, meninges or brain – usual causative agents are falls, motor vehicle crashes and bicycles 2. Pathophysiology: elastic pliable skill of infant and young child absorbs much of direct energy a. directly related to force of impact and secondary forces i. acceleration injury ii. deceleration injury 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 a. may or may not be conscious b. irritable c. pale d. vomiting e. sleepy f. LOC may be altered g. pupils may be affected 4. Diagnostics: assess for pre-existing disorders, take history of event, CAT SCAN, MRI, EEG 5. Management a. acute control ICP: medications: sedating agents, steroids, anticonvulsants, ventilatory support, positioning, HOB up 30° once cervical spine injury is ruled out ii. long-term rehabilitation possible 6. Complications: hemorrhage, infection, cerebral edema and brainstem herniation through the tentorium 7. Nursing interventions a. b. c. d. e. f. neuro and vital signs monitor ICP maintain seizure precautions quiet environment assess LOC level of comfort i.

g. observe for rhinorrhea, otorrhea and test for glucose if drainage present 8. Teaching points a. long-term process b. keep parents informed and teach about the equipment c. teach parent to talk to child but don’t ask them to do things like "squeeze my hand" M. Meningitis: acute inflammation of the meninges and CNS 1. Etiology: can be caused by pneumococcal or meningococcal bacteria (most serious) or can be caused by a virus. 2. Pathophysiology: most common route of infection by vascular dissemination from an infection elsewhere a. organisms then spread into CSF and throughout subarachnoid space b. infective process i. inflammation ii. exudate iii. WBC accumulation iv. tissue damage v. brain becomes hyperemic and edematous 3. Findings a. fever b. chills c. headache d. vomiting e. LOC alterations f. irritable g. nuchal rigidity h. positive Kernig and Brudzinski signs 4. Diagnostics a. lumbar puncture: definitive diagnostic tool, CSF is tested for gram stain, blood cell count and presence of glucose and protein content b. sedation of child may be required. Versed, Demerol or Fentanyl may be used 5. Management: treated as bacterial until causative agent is identified a. bacterial meningitis is an emergency b. respiratory isolation precautions until causative agent identified c. medications: antibiotics 6. Nursing interventions a. monitor fluid status - IV fluids b. monitor electrolytes c. neuro and vital signs d. maintain quiet environment e. monitor for seizures f. assess peripheral vascular collapse if causative agent is meningococci 7. Teaching points a. immunizations are available to prevent several types IV. V. Pediatric Endocrinology The endocrine system A. 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 B. 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

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impulses in the nervous component and by blood in the endocrine system. the two systems function synergistically. Impulses transmitted via the nervous system stimulate the hypothalamus to secrete releasing or inhibiting factors

B. Hypopituitarism 1. Definition: diminished or deficient secretion of pituitary hormones, primarily GH (Somatotropin) 2. Etiology: tumors, hypoplasia, auto-immune, congenital, acquired, irradiation, malnutrition, trauma 3. Pathophysiology: leads to gonadotropin deficiency - inhibits somatic growth in all body cells 4. Findings a. usually dependent on hormone involved and age of onset (dwarfism) b. absence or regression of secondary sex characteristics c. example: slow growth, short stature, normal intelligence; delayed puberty 5. Diagnostics a. family history b. history of growth patterns of child c. MRI d. radioimmunoassay of plasma GH levels e. x-rays of hand and wrist for centers of ossification 6. Management a. depends on cause b. hormone replacement therapy: growth hormone 7. Nursing interventions: a. support child and parents during period of diagnosis b. plan realistic goals with child and family 8. Teaching points a. show parent how to administer daily hormone replacement sc b. educate child and family about long term process C. "Precocious puberty" - unusually early activation of maturation process that is considered normal later in life 1. Definition: manifestations of sexual development before age nine in boys or age eight in girls 2. Etiology: brain lesions, inflammatory disorders, idiopathic, adrenal disorders 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 - occurs most often in girls 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 6. Nursing interventions a. provide support and guidance to parents and child b. child may be embarrassed c. assign nurse of same sex as child d. early identification of affected child 7. Teaching points a. long-term problem

b. after puberty, child will be the same as peers

D. 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 - under 120 mEq/L child becomes symptomatic - lower sodium levels cause neurological findings 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 6. Nursing Interventions: a. assess patients at risk for early identification b. I & O – daily weights c. monitor for signs of fluid overload d. seizure precautions if sodium level low E. Hypothyroidism 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 7. Nursing interventions a. assess children at risk. b. monitor administration of replacement hormone – can’t be increased too fast c. allow rest periods or cluster care d. encourage child to exercise when able 8. Teaching points a. life-long problem

b. child needs periodic blood tests to monitor hormone levels.

F. 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 - Propylthiouracil or Tapazole to decrease amount of circulating thyroid hormone b. surgery (subtotal thyroidectomy) – check voice quality after surgery. 6. Nursing interventions a. assess behavior patterns before and after medication b. quiet non-stimulating environment c. administer moisturizing eye drops for child with exophthalmos d. provide nutrition appropriate for activity level e. establish a routine with child and parents 7. Teaching points a. side effects of propylthiouracil -sore throat and fever b. child may need to reduce activities for a while 8. Concern: thyrotoxicosis or “thyroid storm” from sudden release of hormone: from vigorous palpation or surgery a. can be a life threatening situation b. findings 1. acute onset of severe irritability 2. vomiting 3. diarrhea 4. hyperthermia 5. hypertension 6. tachycardia 7. prostration c. treat with cooling blanket d. propranolol (Inderal), potassium iodine (thyro-block) e. antithyroid drugs G. Diabetes mellitus - type 1 1. Definition: deficiency of hormone insulin - most common endocrine disorder of childhood. 2. Etiology: genetic, auto-immune response; usually with trigger factors of virus, bacterium or possible chemical irritant 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 entering the cells - the concentration in bloodstream increases (hyperglycemia)

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d. when glucose level exceeds kidney's threshold (about 180 mg/dl) kidney then "spills" glucose into urine (glycosuria) e. producing osmotic diureses (polyuria) f. starved for glucose g. urinary fluid loss causes excessive thirst (polydipsia) Findings a. hyperglycemia 1. the three "polys" of diabetes: polydipsia, polyuria, polyphagia 2. additional findings: fatigue, hunger, weight loss, enuresis Diagnostics a. fasting blood glucose b. oral glucose tolerance test c. urine tests for presence of ketones and/or glucose d. glycosylated hemoglobin (A1c) reflects average blood glucose levels for past 2 to 3 months Management a. medications: insulin (rapid-acting-Lispro, short-acting - regular, intermediate-acting-NPH). Insulin needs are affected by food intake, exercise, emotions, growth spurts and illness b. other: diet, age appropriate activity levels. No special foods - need sufficient calories. Must eat snacks - and meals at the same time each day. c. self-blood glucose monitoring d. insulin pump delivers insulin continuously Concerns: "honeymoon period" and insulin regulation, compliance, sick day management, ketones Complications a. DKA (diabetic ketoacidosis) or extreme hyperglycemia (blood sugar >350 mg/dl) 1. etiology: not enough or no insulin - body chooses alternate source of energy - fat 2. findings 1. fruity breath, decreased level of consciousness 2. nausea/vomiting, abdominal pain 3. 10 percent dehydration 4. increased urine output 5. Kussmaul's respirations 6. metabolic acidosis b. management 1. place on cardiac monitor 2. regular insulin (IV drips and then subcutaneous) 3. frequent monitoring of blood glucose 4. frequent monitoring of electrolytes; neuro checks Hypoglycemia a. etiology: most common cause of insulin therapy and bursts of physical activity, without additional food or with missed meals before meals or when insulin is peaking b. findings 1. fatigue 2. nervousness 3. pallor 4. sweating 5. palpitations 6. hunger 7. loss of coordination 8. seizures 9. coma c. management 1. 10 to 15 mg f simple carbohydrate - honey of low fat milk 2. followed by complex carbohydrate such as slice of bread or crackers

3. occasionally glucagons is prescribed - for home treatment works in about 10 minutes 10. Nursing interventions a. assess child frequently neuro and vital signs b. blood glucose levels c. administer insulin as ordered d. support child family e. arrange meetings with team members - dietary, PT, and social worker 11. Teaching points a. family may be overwhelmed with diagnosis b. use teaching aids to facilitate their success - booklets, videos c. illness management d. insulin should never be omitted e. dose may change f. fluid balance is a concern V. Pediatric Gastrointestinal

A. Dehydration

1. Definition: occurs when total output of fluid exceeds total intake a. 60% of fluid lost from extracellular fluid (ECF) b. 40% of fluid lost from intracellular (ICF) c. insensible fluid loss – lost through skin, urinary loss, fecal loss, and respiratory tract 2. Differences compared to adults 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 kilogram 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. Primary form of dehydration in children. 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 b. leads to eventual loss of fluid from ICF c. cellular dysfunction, shock 5. Nursing interventions a. fluid losses must be replaced b. monitor urine output and specific gravity, daily weight c. fontanels in infant d. electrolyte losses: monitor sodium, potassium, chloride, calcium e. acid-base balance 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) 2. Vomiting 7. Definition: forceful ejection of gastric contents through the mouth 8. Etiology: infection, obstruction, allergy, psychological causes, motion sickness, neurologic lesions, and inflammatory process 9. Pathophysiology a. cause stimulates emetic center of brain b. mechanism of vomiting involves autonomic nervous system 1. salivation, sweating 2. pallor, increased heart rate 3. contraction of stomach antrum & duodenum c. types of vomiting: regurgitation, forceful, projectile 10. Management a. detect and treat the underlying cause b. prevent dehydration, electrolyte loss and acid-base disturbance c. provide anti-emetic medications if needed 11. Nursing interventions a. assess vomitus b. assess child for dehydration c. keep child NPO d. position child to avoid aspiration when vomiting e. monitor electrolytes f. encourage child to brush teeth or rinse mouth after vomiting 12. Teaching points a. teach parents to start feeding child slowly with clear fluids when tolerated

Upper GI Disorders C. 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. Pathophysiology: most common a. TEF – proximal esophageal segment terminates in a blind pouch b. distal segment is connected to trachea or primary bronchus 4. Findings a. coughing b. cyanosis with feeds c. increased oral secretions d. depend on type of defect e. choking 5. Diagnostics: history, chest and abdominal x-rays 6. Management a. pre-op: airway patency, NPO, IV therapy, place in position least likely to cause aspiration b. prevention of aspiration pneumonia c. surgical emergency 7. Nursing interventions a. assess each newborn for the malformation b. intermittent suctioning c. position - head of crib elevated 30 degrees d. care of gastrostomy tube e. tube feedings are begun when tolerated f. observe initial oral feeding – sterile water 8. Teaching points a. teach parents the care of the gastrostomy tube and how to administer feedings b. findings of respiratory distress c. suctioning techniques if appropriate D. Gastroesophageal reflux disease: GERD

1. Definition: transfer of gastric contents in the esophagus 2. Etiology: physiological. functional, pathologic – very common – If GERD occurs often and findings persist, treat it – hiatal hernia and coughing may lead to GERD 3. Diagnostics: history, barium swallow, upper GI series, esophageal pH, manometry studies, endoscopy, scintigraphy – detects radioactive material in esophagus after feeding of the material and also assesses gastric emptying 4. Management a. depends on the severity of findings b. none if child is thriving without findings c. diet: thicken feeds, decrease caffeine intake, 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 (Tagamet or Pepcid), proton pump inhibitors (Nexium or Protonix) f. surgery: Nissen fundoplication 5. Nursing interventions a. monitor feeding position and feeding tolerance b. monitor nutritional status c. check I & O d. monitor electrolyte levels 6. Teaching points a. teach parents to have child avoid caffeine, chocolate, and spicy foods b. older children should avoid tobacco and alcohol c. medication administration schedule should be followed to receive optimal benefit E. Pyloric stenosis 1. Definition: circumferential muscle of the pyloric sphincter becomes thickened resulting in elongation and narrowing of the pyloric channel 2. Etiology a. unknown cause b. hypertrophy, hyperplasia of circular muscles of pylorus 3. 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 4. 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 5. Diagnostics: history, abdominal x-ray, upper GI, ultrasound, electrolytes 6. Management a. pre-op: IV fluids, NPO, surgery (pyloromyotomy) also called Fredet-Ramstedt Procedure b. post-op: small, frequent feedings initially, gradually increasing 7. Nursing interventions a. pre-op 1. monitor IV fluids and electrolyte replacement 2. document vomiting episodes and stools 3. access patency of N/G tube if present 4. position child flat or head slightly elevated b. post-op

1. monitor for vomiting 2. assess IV sites 3. begin feeding clear liquids with glucose and electrolytes 4. assess operative site for drainage or inflammation 8. Teaching points a. child may vomit after surgery b. this disorder may run in families c. child will be discharged when able to tolerate feedings Lower GI Disorders F. Constipation 1. Definition: infrequent passage of firm or hard stools 2. Etiology a. triggered by diet, medication, dehydration, emotions, or neurogenic b. structural disorders (Hirschsprung's disease)

c. systemic disorders (hypothyroid) 3. Findings a. abdominal pain and cramping b. palpable, movable fecal mass c. malaise, anorexia, nausea 4. Diagnostics: abdominal x-rays, rectal exam, palpation and percussion 5. Management a. prevention: higher fiber diet, fluids, exercise, regular toileting habits b. medications: stool softeners, enemas, laxatives 6. Nursing interventions a. assess child’s usual pattern of bowel elimination b. provide dietary modifications that promote bowel elimination appropriate for age c. establish bowel routine for parents

7. Teaching points a. dietary modifications b. bowel routine G. 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 H. Celiac disease 1. Definition: gluten-sensitive enteropathy (GSE) = (celiac sprue) 2. Etiology: absorption problem with genetic predisposition, possibly immune abnormality 3. Pathophysiology a. inability to digest gliadin (byproduct of gluten); permanent intolerance of gluten b. increasing levels of glutamine in the small intestine, toxic to mucosal cells c. atrophy of villi and decreased absorptive surface

d. malabsorption of fats, carbohydrates, vitamins and electrolytes

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e. gluten is found in the grain of wheat, barley, rye, and oats 4. 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 5. Diagnostics a. history (symptoms occur three to six months after infant begins eating grains) b. serum anti-gliadin antibody (AGA) c. jejunal biopsy - reveals mucosal inflammation, villous atrophy and crypt hyperplasia d. serologic testing to detect antibodies 6. Management a. diet: gluten-free with vitamin supplements b. crisis: IV fluids, steroids 7. Nursing interventions a. monitor tolerance of new diet – weight gain b. monitor episodes of diarrhea c. assess for steatorrhea d. provide diet high in calories – fruits, vegetables – low fat 8. Teaching points a. gluten free diet restrictions b. reading food labels Intussusception 1. Definition: one of the most frequent causes of intestinal obstruction between ages of three months and five years 2. Etiology: unknown – possible hypertrophic response to the virus; is common in children with cystic fibrosis 3. Pathophysiology a. trigger – bowel (proximal segment) “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 4. Findings a. sudden acute abdominal pain b. bilious vomiting c. currant jelly stools d. sausage-shaped abdominal mass e. lethargy 5. Diagnostics: barium enema – abdominal x-ray, rectal exam reveals mucus and blood 6. 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 7. Nursing interventions a. monitor fluid status b. assess pain c. monitor for barium excretion and passage of brown stool that indicates intussusception has resolved d. usually post operative care if child has surgery 8. Teaching points

a. parents may feel guilty they didn’t know about child’s condition b. everything moves fast c. child may need hospitalization J. 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 – reveals absence of ganglion cells 5. Management a. surgical correction: remove aganglionic portion b. temporary ostomy for three to six months, then reanastomose 6. Nursing interventions a. assess fluid and electrolyte status b. monitor comfort level c. assess colostomy d. provide nutrition as ordered e. assess N/G f. make referrals for home care assistance 7. Teaching points a. colostomy care – as child will be discharged b. parents should be told that toilet training may be difficult K. Appendicitis

1. Definition: inflammation of vermiform appendix (blind sac at end of cecum)

2. Etiology a. virus, hardened fecal material, parasites, foreign body b. most common in school-aged children 3. Pathophysiology a. trigger obstruction increased mucus accumulation b. distention, capillary occlusion and engorgement of capillary walls c. eventually, abscesses and fistula form d. possible perforation leading to peritonitis 4. Findings a. colicky abdominal pain 1. generalized but usually localizes to lower right quadrant 2. most intense pain at McBurney's point b. nausea, vomiting c. possible fever and chills d. decreased bowel sounds e. guarding of abdomen - stooped posture f. rebound tenderness 5. Diagnostics: physical exam, laboratory tests (CBC with WBC), MRI, possible also C Reactive Protein (CRP) a. may order HCG serum human gonadotropin on adolescent females to rule out ectopic pregnancy 6. Management a. surgical removal (open or laparoscopic) b. IV fluids c. antibiotics d. analgesics 7. Nursing interventions a. postoperative 1. monitor IV fluids – comfort level 2. position in semi Fowlers 3. check drainage tubes if present 4. prevent fluids as bowel sounds return if ordered 8. Teaching points a. activity level at home b. when can return to school, if known c. caution: do not give enemas or cathartics or use heating pad if suspected appendicitis

VI.

Genitourinary System

A. Common findngs: renal and urinary tract (UT) disorders Renal Findings: -Urination (polyuria, oliguria, urgency) -Fluids (increased BP, Edema) UT Findings: -2 Clusters G-I (Anorexia, Nausea/vomiting, Thirst/dehydration, Failure to thrive) -Lower Body (Abdominal & back pain, Painful or frequent urination, Persistent diaper rash) 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 a. bacterial (E. coli. - 80% of cases), structural defect, physiological

b. extrinsic (example: Foley catheter, medications) c. improper perineal hygiene 3. Pathophysiology a. organism usually ascends through urethra to bladder b. with a structural defect, urine flows back from ureters into kidney 4. 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 5. Diagnostics: urine culture and serum testing, radiographic testing 6. Management a. antimicrobials b. antipyretics c. analgesics d. fluids (IV and oral) 7. Nursing interventions a. careful history b. check diaper every half-hour c. provide adequate or increased fluid intake d. monitor for irritability 8. Teaching points a. hygiene – wipe front to back for girls b. do not delay urination c. if child is maintained on daily low dose antibiotics – giving dose at bedtime allows medications to remain in bladder overnight C. Enuresis 1. Definition: inability to control bladder functions, often due to maturation; nocturnal, or "bed wetting" very common 2. Etiology 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 - bladder training iv. medications 1. tricyclic antidepressant; imipramine (Tofranil) 2. desmopressin (DDAVP) b. secondary: treat underlying cause 5. Nursing interventions a. help parents understand problem and treatment plan b. involve child in planning, if age appropriate 6. Teaching points a. do not punish child if enuresis occurs Upper Urinary Tract Disorders D. Vesicourethral reflux (VUR) 1. Definition: retrograde flow of bladder urine into the ureters during voiding 2. Etiology

a. primary (congenital anomaly) - abnormal insertion of ureters into bladder 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 1 to 5 4. Findings: UTI with chronic findings and recurrences 5. Diagnostics a. radiographic studies b. voiding cystourethrogram c. urine culture 6. Management a. medications: for grades 1 to 4, low-dose antibiotics; monitor b. surgery: for severe cases, grade 4 or grade 5 7. Nursing interventions a. adequate or increased fluid intake b. usual post-op care if child has surgery c. monitor I & O 8. Teaching points a. all children in family should be screened for possible reflux E. Acute glomerulonephritis (AGN) 1. Definition: immune complex disease that occurs after a streptococcal infection (A B-hemolytic strep) 2. Etiology a. previous acute bacterial infection (pharyngitis, impetigo) b. underlying systemic disorder 3. 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 4. 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 5. Diagnostics: urine testing, serum (antibody, complement, CRP, ESR, WBC), throat culture, history of antecedent strep infection 6. 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. nutrition (low in sodium, protein, potassium) 7. Nursing interventions a. monitor vital signs, fluid balance, and behavior b. assess child’s appearance – presence of edema c. possible institution of seizure precautions if indicated d. possible fluid restriction e. provide frequent rest periods 8. Teaching points a. importance of finishing medications as ordered

b. findings of recurrence F. Chronic nephrosis (nephrotic syndrome) 1. Definition: 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: mainly occurs in children between 2 and 7 years-old 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 - urine smoky brown (resembles tea or cola) g. lethargy h. pallor 5. Diagnostics: history, urine tests will show massive proteinuria, BUN and creatinine will be elevated, and antistreptolysin O(ASO) titer 6. Management – mainly supportive of what child demonstrates a. diet: no added salt but high in potassium and protein b. medications: corticosteroids, immunosuppressants, diuretics 7. Nursing interventions a. monitor vital signs and body weight and I&O b. assess volume and character of urine c. restrict fluid intake d. seizure precautions if appropriate e. activity should increase as protein in the urine decreases f. place child in semi-Fowler’s or Fowler’s to treat periorbital edema 8. Teaching points a. how to test urine at home for presence of protein b. dietary restrictions c. findings of infection G. Wilm’s tumor 1. Definition: most common renal and intraabdominal tumor of childhood 2. Etiology: probably arises from malignant undifferentiated cluster of cells – favors left kidney 3. Pathophysiology a. cells regenerate an abdominal structure – renal function is impaired 4. Findings a. abdominal mass or swelling – firm, nontender b. fatigue, malaise c. respiratory findings if metastasis 5. Diagnostics

a. chest and abdominal x-rays b. laboratory tests – CBC 6. Management a. surgical removal – possible chemotherapy, actinomycin D and radiation 7. Nursing interventions a. pre-op - post signs on bed "do not palpate abdomen" b. post-op - assess BP, urine output and pulmonary status 8. Teaching points a. support family b. child should avoid contact sports c. findings of infection H. Hemolytic uremic syndrome (HUS) 1. Definition: most common acquired renal failure in children 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 – fresh frozen plasma (FFP) c. for renal symptoms: fluids, possible dialysis or peritoneal dialysis for the child who has been anuric for 24 hours d. treatment of hypertension e. correction of electrolyte disorders 7. Nursing interventions a. monitor vital signs b. monitor I&O c. prepare for dialysis d. monitor laboratory results e. monitor transfusion response 8. Teaching points a. possible renal impairment b. keep parents informed of treatment plan as everything happens quickly I. Acute renal failure 1. Definition: sudden, severe loss of function of kidneys, usually reversible 2. Etiologies a. dehydration b. underlying conditions c. burns (thermal injuries) 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 b. acute: fluids, electrolytes, dialysis (peritoneal, hemodialysis), antihypertensives, diuretics 7. Nursing interventions a. monitor level of dehydration and volume restoration to avoid water intoxication and hyponatremia b. insert Foley c. monitor laboratory results d. assess potassium in food and fluid e. monitor vital signs especially blood pressure f. neuro signs, if appropriate 8. Teaching points a. nutritional needs and appropriate snacks b. include parents when formulating plan of care c. possible complications: acute and chronic renal damage, infection and cardiac failure J. 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. Nursing interventions a. monitor body fluid volume and electrolyte balance b. monitor vital signs, especially blood pressure c. monitor ABG’s for metabolic acidosis d. monitor rate of growth and development e. assess for findings of infection f. prepare child and parents for possible dialysis and/or transplantation 8. Teaching points a. signs of rejection of new kidney b. need to follow treatment plan and take medications as ordered 9. Concerns: bone demineralization, anemia, failure to thrive, retention of wastes; transplant concern Genitalia Problems K. 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 (older child), or surgery (orchiopexy). L. 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 postop), post-op pressure dressing must not be removed by anyone other than the health care provider, usually repaired before child goes to school 5. Concerns: stenosis, chordee, body image/self esteem M. Bladder exstrophy 1. Definition: externalization of bladder, splaying at the urethra with failure of tubular formation and diastasis of pelvic bone 2. Etiology: congenital - associated with genital abnormalities 3. Pathophysiology a. failure of abdominal wall and underlying structures, including the ventral wall of the bladder, to fuse in utero b. bladder develops outside c. the earlier in gestation, the more severe the defect d. defect almost always associated with epispadias 4. Management a. treament objectives 1. preservation of renal function 2. attainment of urinary control 3. adequate reconstructive repair 4. preservation of optimum sexual function b. pre-op 1. prevent organs from drying out, fluids, infection control 2. surgery: staged procedure c. post-op (antibiotics, compression bandage, bed rest) 5. Concerns: urinary incontinence, VUR, infection N. Ambiguous genitalia 1. Etiology: abnormalities in chromosomal complement, embryogenesis, or hormones 2. Pathophysiology: interruption in normal development around seven to eight weeks gestation, when normally male begins differentiating from female

3. Diagnostics: history, physical exam, tests to determine gender (endoscopy, ultrasound, radiographic contract), genetics lab test, laparotomy, biopsy 4. Concerns: body image/self esteem, family support

VII.

Pediatric Musculoskeletal System

General Concepts: A. Children's musculoskeletal differences 1. Bones are more pliable and porous; bend, buckle, absorb shock 2. Tendons and ligaments are more flexible 3. Bones produce callus that speeds healing 4. Thicker periosteum, so stronger and more active osteogenesis 5. Skull is pliable during infancy; anterior fontanel fuses at 18 months; posterior fontanel fuses at two months 6. Skeletal maturation completes when epiphysis fuses with diaphysis, which is usually 18 to 21 years of age B. Immobilization in children 1. Affects multiple systems 1. muscular, GI, GU, pulmonary, cardiovascular, integumentary 2. psychologic, behavioral, economic 2. Affects normal growth and development 3. Pathologic changes occur due to 1. decreased muscle strength and mass, decreased metabolism 2. possible bone demineralization 3. decreased range of motion and decreased joint movement 4. Concerns 1. hydration 2. dietary modification 3. activity as condition permits 4. self care as condition permits

5. play/exercise C. Fractures in children 1. Definition: break or disruption in bone continuity 2. Etiology: usually due to mobility and immature motor and cognitive skills, trauma, osteogenic diseases, birth injuries, child abuse 3. Pathophysiology 1. fractures are seldom complete breaks (bone is flexible) 2.

3. many types: greenstick, spiral (may indicate child abuse), oblique, transverse, comminuted, pathologic 4. classification: simple (closed) or compound (open); complete or incomplete 5. pediatric risks 1. external hemorrhage creates risk of critical blood loss 2. break at epiphyseal plate (growth plate between epiphysis and metaphysis) may affect future limb growth

4. Findings: swelling, pain, bruising, edema, muscle rigidity, diminished functional use of affected part 5. Diagnostics: x-rays, laboratory testing 6. Management: cast or traction to realign, possible surgery

7. Concerns: proper bone healing and alignment, neurovascular status, pain, bone growth, compartment syndrome

Structural Musculoskeletal Disorders

D. Clubfoot

1. Definition: congenital malformation of one or both feet 2. Etiology: 95% of cases talipes equinovarus - foot is pointed downward and inward 3. Findings: plantar-flexed foot/feet, with inverted heel and adducted forefoot 4. Management: serial manipulation and casting after birth; possible surgery E. 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 F. Hip dysplasia 1. Definition: developmental dysplasia of the hips (DDH), or dislocation 2. Etiology: congenital, pre- and post-natal positioning 3. Pathophysiology: head of femur is improperly seated in acetabulum in hip

4. Findings 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 5. Diagnostics a. physical exam/screening at birth and monitor for hip dysplasia throughout first year of life b. check for Ortolani maneuver and Barlow's test from birth to 2 or 3 months of age c. radiographic studies 6. Management: Pavlik harness, spica cast, traction, surgery, Bryant's traction 7. Concerns: compliance, skin integrity, avascular necrosis from improper positioning of harness G. Scoliosis

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, Wilmington and Boston orthoses - plastic shell, or TLSO - custom-molded jacket) b. if curve progresses, surgery - arthrodesis - surgery is quite complex 7. Concerns a. body image/self esteem, pain and discomfort - long term b. compliance with exercises and bracing; skin integrity, airway clearance Infectious Musculoskeletal Conditions H. Osteomyelitis 1. Definition: bacterial bone infection 2. Etiology: endogenous (in-body) sources, or direct entry via open fracture or external fixation devices. Staphylococcus aureus is the most common pathogen 3. Pathophysiology a. microbe affects metaphysis of long bone; inflammation b. pus forms and spreads along the shaft of the bone c. new bone starts to form, but existing cortex loses blood vessels and necrotic area will detach (sequestrum) d. "honeycombed" areas contain infective material 4. Findings a. pain, localized tenderness, erythema over involved bone b. decreased range of motion at affected bone c. irritability, restlessness, fever 5. Diagnostics a. laboratory: blood cultures and bone aspirate cultures i. erythrocyte sedimentation rate (ESR) will increase ii. increased white blood cells iii. blood culture b. radiographic studies (often negative for ten to 14 days); bone scan 6. Management a. IV antibiotics; may require long-term IV access (4 to 6 weeks) b. bed rest c. possible immobilization with splints or cast d. possible surgery 7. Nursing interventions a. monitor antibiotic levels b. monitor level of comfort c. perform range of motion if possible – no weight bearing d. monitor nutrition – high calorie liquids e. provide diversional activities for age 8. Teaching points a. may continue IV therapy at home b. physical therapy at home c. school work at home if appropriate d. play activities within limits of physical ability Other Musculoskeletal Disorders I. Juvenile rheumatoid arthritis (JRA) - new name Juvenile Idiopathic Arthritis 1. Definition: inflammation of joints 2. Etiology: auto-immune with probable genetic predisposition 3. Pathophysiology

a. trigger inflames synovium - chronic inflammation
b. effusion of the joint and increased fluid c. erosion and fibrosis of the articular cartilage d. further deterioration occurs with bone erosion e. decrease in joint's range of motion and function 4. Findings a. may have sudden inability to walk on one leg b. intermittent joint pain, stiffness, swelling c. decreased range of motion, morning stiffness d. significant disability 5. Diagnostics a. laboratory testing - no definitive serologic tests 1. increased ESR and CRP: c-reactive protein (sign of rheumatic fever) 2. anemia 3. leukocytosis in early stages 4. HLA testing (human leucocyte antigen) b. radiographic testing and x-rays -- widening of joint spaces followed by gradual fusion and articular destruction with soft tissue swelling 6. Management a. treatment objectives 1. preserve joint function 2. prevent physical deformities 3. relieve findings b. medications: nonsteroidal anti-inflammatory drugs (NSAIDS) naproxen, slow acting antirheumatic drugs (SAARDS), corticosteroids, immunosuppressives, cytotoxic agents methotrexate c. other: PT and OT, rest/activity, positioning, splinting, ultrasound, electrical stimulation, heat, whirlpool; surgery for joint replacement 7. Nursing interventions a. monitor for compliance with therapy – physical and occupational b. prevent contractures c. monitor pain/discomfort – possible application of moist heat d. provide family and child support services, body image, decreased mobility e. assess for altered patterns of growth and development due to decreased activity, decreased ability to perform ADL’s 8. Teaching points a. multidisciplinary approach b. long-term disorder c. usual developmental milestones – age appropriate toys d. exercise needs J. Legg-Calve-Perthes disease: osteochondritis 1. Definition: aseptic necrosis of femoral head 2. Etiology: disturbance of circulation to the femoral epiphysis 3. Pathophysiology a. disturbance in blood supply b. ischemic aseptic necrosis of femoral head, usually self-limiting

4. Findings a. insidious onset b. intermittent painful limp on one side c. increased pain after extended period of activity d. decreased range of motion 5. Diagnostics: history, radiographic studies, bone scan, MRI 6. Management a. bed rest b. non weight bearing range of motion c. corrective devices such as braces, casts, traction – conservative therapy must be continued for 2 to 4 years d. possible surgery 7. Nursing interventions a. mostly outpatient activities 8. Teaching points a. parents must learn purpose, function, application, and care of corrective devices b. play and exercise activities for a child who feels well but must remain relatively inactive

VIII.

Pediatric Integumentary System

General Concepts A. Pediatric differences: thinner skin 1. Newborn's skin thinner, more easily hurt by endotoxins and infection, shearing and tearing forces 2. Child's body absorbs more of topical products 3. Harder to regulate body temperature B. Factors in skin condition 1. Age 2. Hydration and nutrition 3. Soap, laundry detergents, and topical products 4. Medications 5. Infectious processes (viral, bacterial, fungal) 6. Mechanical forces (tearing, friction, shearing) & vascular damage 7. Tape and adhesive products 8. Allergy 9. IV infiltration 10. Temperature 11. Bodily secretions: urine, stool, ostomy drainage, fistula C. Wounds, wound healing, modes of skin care 1. Assessment a. acute or chronic: etiology of pressure, shearing, trauma b. measurements: depth, size, location, "tunneling", open, closed c. staging: partial or full thickness, complex (involving muscle, bone) 2. Factors impacting wound healing

a. b. c. d. e. f.

perfusion, oxygenation nutritional deficiencies infection underlying systemic condition topical products utilized medications

3. Principles of successful wound management a. remove or eliminate causative factors b. provide appropriate systemic support: fluids, nutrition c. apply appropriate topical products: encourage moist wound healing and process of occlusion. 4. Evaluation of successful wound healing a. type: primary, secondary or tertiary wound closure b. measurement: decreasing size, viable tissue, decreased exudate, color 5. Modes of caring for skin disorders a. non-surgical: skin care products, nutrition, healthy lifestyle b. surgical: debridement, skin grafts and flaps c.

Noninfectious Skin Conditions D. Atopic dermatitis (eczema)

1. Definition: inflammation of the skin 2. Etiology: genetic tendency, multifactorial with family history of allergies or asthma; exacerbated by stress and certain foods 3. Pathophysiology: trigger- increased histamine release with inflammatory response - itching and findings of exacerbation 4. Findings a. dry skin, itching, erythema b. macule, papule, pustule and even vesicles possible c. acute weeping areas with excoriated red plaques 5. Diagnostics a. family history b. physical exam c. lab tests (eosinophils, IgE) 6. Management a. goals: control itching, moisturize, remove irritants and allergens, and prevent secondary problems (infections) b. products: wet compresses, occlusive dressings, creams/ointments, hydrocortisone cream, mild detergents c. medications: antihistamines 7. Nursing interventions a. remove allergens b. keep child’s fingernails short c. clothe lightly to decrease sweating d. apply emollient preparation immediately after bathing e. provide rest periods f. provide hypoallergenic diet if appropriate 8. Teaching points a. provide family support regarding nutrition and recurrent nature of this disorder E. Seborrheic dermatitis (in infants: cradle cap) 1. Definition: recurrent inflammatory reaction of skin that may also involve the eyelids and external ear canal 2. Etiology: probable dysfunction of sebaceous glands 3. Findings: usually nonpruritic oily scales on scalp, forehead and eyebrows or behind ears 4. Management: scalp hygiene with mild baby shampoos, hydration creams, baby oil 5. Teaching points: teach parents how to clean scalp and shampoo hair

F. Diaper dermatitis ("diaper rash")

1. Definition: inflammatory skin disorder caused directly or indirectly by wearing of diapers 2. Etiology a. ammonia in urine, fecal enzymes, detergents, moisture, heat b. can lead to secondary fungal infection (especially candida albicans) 3. Pathophysiology a. prolonged and repetitive contact with irritant (especially urine ammonia, which is formed by urea breakdown from fecal bacteria); inflammation b. excoriation, with macules/papules and erosion c. concern: secondary infection. 4. Findings: red, excoriated, macules/papules, maceration 5. Management a. cleaning and frequent diaper changes b. skin protectants/moisture barriers to keep skin dry c. topical antifungals and steroids d. if diarrhea, treat underlying cause G. Contact dermatitis

1. Definition: inflammatory reaction of the skin to chemical substances natural or synthetic 2. Etiology: multiple factors and irritants foods, solutions, allergens, plants 3. Findings: irritant and allergic types a. irritant: causes dry, inflamed, pruritic lesions where irritant touched b. allergic: blisters after weeping, pruritic, lesions 4. Management: topical (antiinflammatory, antipruritic) cold compresses, supportive care – prevent further exposure to offending substance if possible H. Acne vulgaris 1. Definition: most common skin problem treated by doctors during adolescence 2. Etiology: multifactorial (heredity, hormones, emotions) 3. Pathophysiology a. puberty – increased androgens – involves hair follicle and sebaceous gland complex b. increased sebaceous glands secrete more sebum c. pores become plugged and dilated d. fatty acids are oxidized on skin and form blackheads OR e. internal fatty acids form whiteheads f. rupture causes local inflammation, sometimes with pustules 4. Findings: whiteheads (closed comedomes), blackheads (open comedomes), papules, pustules, nodules, red and excoriated skin 5. Management a. general: good cleansing of skin, nutrition, and no squeezing or picking at lesions b. topicals: vitamin A, topical antibiotics, topical benzoyl peroxide c. medications: oral antibiotics and possible estrogen for girls 6. Teaching points a. possibility of scarring b. possible bacterial invasions c. body image d. education needed on myths and realities of causative agents and treatment modalities e. stress importance of compliance to treatment program Infectious Disorders I. Impetigo contagiosa 1. Definition: bacterial infection of skin 2. Etiology: staphylococcus aureus, group A beta-hemolytic streptococcus 3. Pathophysiology: starts in area of broken skin - highly contagious for seven to ten days 4. Findings a. primary: macules-vesicles of bulla that rupture

b. secondary: honey-colored crusts, superficial erosion, easily bleeds, pruritic c. tends to heal without scarring unless secondary infection 5. Management a. careful removal of crusts/debris with warm soapy solution or Burow's solution b. topical antibiotics for early small lesions c. treatment of choice: systemic antibiotics d. short fingernails 6. Concerns: highly contagious (good hand washing, no sharing towels or eating utensils) J. Cellulitis 1. Definition: infection of dermis and/or subcutaneous tissue 2. Etiology: strep, S. aureus, Haemophilus influenza 3. Pathophysiology a. starts in area of broken skin b. highly contagious for days c. can occur on any part of the body 4. Findings a. affected area (red, edema, tender, occasional discoloration) b. enlarged lymph nodes, "streaking" frequently seen, fever, malaise, headache 5. Diagnostics: CBC, cultures, possible skin culture, CT 6. Management: oral or parenteral antibiotics, warm moist compresses, possible incision and drainage, rest of affected area and child 7. Concerns: secondary infection, alteration in skin integrity K. Herpes simplex type one

1. Definition: oral herpes, cold sore, fever blister 2. Etiology: HSV-1 or HSV-2, cytomegalovirus (CMV), Epstein Barr (infectious mononucleosis), Varicella zoster 3. Pathophysiology: virus infects body fluids, which then come in contact with breaks in the skin or mucous membranes a. HSV-1 affects areas above the waist; "cold sore" b. HSV-2: affects areas below the waist; genital c. virus dormant within nerve cells; then reactivated by fever, stress, trauma, sun exposure, menstruation 4. Findings a. often depend on location in body b. prodromal period common c. vesicles, pain, pruritis, paresthesias, increased skin sensitivity at site 5. Diagnostics: history, physical exam, smears, tissue cultures (Acyclovir Valtrex) 6. Management - symptomatic: fluids; possible antiviral or antibiotic if secondary infection; analgesics 7. Concerns: highly contagious, secondary infection, pain, body image; HSV-2 is sexually transmitted Fungal Infections of Skin L. Moniliasis candidiasis (thrush) 1. Definition: oral candidiasis characterized by white adherent patches on the tongue, palate, and inner aspects of the cheeks 2. Etiology: fungus, candida albicans 3. Pathophysiology: acquired via birthing process, antibiotics, inhaled steroids 4. Findings a. white plaques on tongue, gums or buccal mucosa b. possible ulcerations in oral mucosa c. itching 5. Management a. oral antifungals (swish and swallow) after feeding - topical application also b. education on mouth rinsing after inhaled steroid 6. Concern: may spread to groin, buttocks

M. Tinea (ringworm)

1. Definition: superficial infection that lives on, not in, the skin 2. Etiology: dermatophytes 3. Pathophysiology a. transmitted person to person, animal contact, contact with contaminated fomites (nonliving "host")

b. associated with poor hygiene, friction from tight clothing

4. Nursing interventions a. compliance, good hygiene b. contagion, teach client never share clothing/hair brushes c. overuse of OTC products, especially for feet 5. Teaching points a. maintain medication schedule as ordered – maybe for several weeks or months b. no sharing of towels, linens or hair brushes Infestations N. Pediculosis (lice)

1. Definition: lice infestation

2. Etiology: infestation of the scalp - pediculus humanus capitis (parasite) 3. Pathophysiology: a. these lice live only on humans, are transmitted by direct and indirect contact b. types include: scalp (capitis), body (corporis), pubic area (pubis) and eyelashes c. all pierce skin and suck blood, with females laying eggs (nits) at the base of hair shaft 4. Findings a. nits (tiny silvery or grayish-white specks) and pruritis b. with corporis, papular rose-colored dermatitis 5. Management a. topical medicated shampoos or body ointments - Nix (creme rinse) for infants and children b. teach client to prevent recurrence and spread: wash clothes in hot water, no sharing of clothes or hair brushes 6. Concerns a. compliance and recurrence b. lindane (Kwell) shampoo is neurotoxic in children under five c. lice are highly contagious; in hospital, infested client requires contact isolation. For information on isolation see Lesson 2: Safety and Infection Control O. Scabies (itch mite)

1. Definition: mite infestation 2. Etiology: mite (sarcoptes scabiei) 3. Pathophysiology a. female mite burrows into epidermis, lays eggs b. mites, eggs and excrement all cause intense pruritis (due to histamine release) 4. Findings a. pruritis, burrows (fine, grayish brown threadlike lines) b. papule like eczema in infants 5. Management a. scabicide topicals, creams - Elmite is the medication of choice b. anti-steroidal agents may be included c. wash clothes and bedding in hot water d. all persons in close contact with affected child will need treatment IX. Temperature-Related Disorders A. Caused by cold 1. Definition a. Types

I.

B.

C.

II. Findings a. chilblain: redness, edema of hands and feet, may burn or itch b. frostbite I. blanching, decreased sensation, mottled II. second degree (cold after rewarming): blisters and possible bulla III. third degree: cyanosis, mottling- then red with swelling, local necrosis, hemorrhagic vesicles IV. fourth degree: complete necrosis, gangrene, loss of body part c. hypothermia: cooling of the body's core temperature to injurious levels (below 35 degrees Celsius) 3. Management a. cover area immediately but no massaging b. rewarm in 100 to 108 degree Fahrenheit water - immerse affected part c. give analgesics and sedatives for severe pain during rewarming d. hypothermia: heat lamps, immersion in water bath Sunburn 1. Definition: overexposure to the sun 2. Etiology: overexposure to ultraviolet light waves UVA (minor burning) UVB (tanning, burning, harmful effects) 3. Factors influencing degree of burn a. genetic makeup and skin type b. season of year, altitude, time of day c. window or glass, light reflected by snow or water d. medications, underlying conditions, topical products 4. Prevention a. avoid sun b. wear good clothing with a tight weave and eye wear c. apply child appropriate topical sunscreens and blocks liberally d. no direct sun between 10 am to 3 pm 5. Management: like a first degree burn (see next page) Thermal injuries (burns) 1. Definition: injuries to skin resulting from extreme heat sources 2. Etiologies: thermal, chemical, electrical, secondary to irradiation; thermal types most common in children 3. Pathophysiology a. severity affected by: location, concurrent inhalation injury, trauma, underlying condition, age of child I. hot water scalds - toddler II. flame related - older children b. anatomy and physiology of burns I. depth I. amount of local tissue destroyed or damaged II. related to length of exposure and temperature II. types: first, second, third, fourth degree 2.

chilblain: skin (usually extremities) becomes red and swollen when it is intermittently exposed to cold temperatures frostbite: tissue damage when ice crystals form in tissue

4.

5.

6.

7.

8.

I. partial thickness: first and second degree II. full thickness: third and fourth degree III. fourth degree: involves muscle, fascia and bone. Wound appears dull and dry and ligaments, tendons and bone may be exposed. IV. severity: classified as minor (<10% TBSA), moderate (1020% TBSA) or uncomplicated major (>20% TBSA); determined by the amount of systemic disruption beyond what the body could normally compensate. c. extent of burn determines responses: local or systemic Local response a. cellular damage and fluid movements - edema b. fluid leaks into interstitial spaces, fluid lost to air c. fluid is lost to circulating volume (the "oliguric" phase) d. burn damages tissue Multi-systemic response and potential complications a. cardiovascular: dehydration- "burn shock" b. pulmonary: respiratory distress, possible post-inhalation injury, ARDS, aspiration pneumonia, pulmonary edema c. GI: ischemia - decreased bowel sounds, possible ileus, Curling's ulcer d. GU: decreased fluids, increasing BUN and creatinine e. metabolic: increased basal metabolic rate, vital signs f. neuroendocrine: increased ADH and aldosterone g. CNS: possible encephalopathy, seizures, coma, altered LOC h. integumentary: burned, infection, scar tissue formation and poor healing i. anemia: associated with major burns Burn injuries differ in children a. thinner skin, so tissue damage is more severe b. fluid volume changes faster than cardiovascular system can respond c. relatively large surface area increases risk for losses of fluid and heat d. increased risk for dehydration and acidosis due to: diarrhea, insensible fluid loss, and because child's body requires higher proportion of water than adult's e. immature immune system increases risk of infection f. long term: scars mature more slowly and keloids more severe Findings a. superficial (first degree) burn: localized pain, dry surface, blanches with pressure, redness, possible blister b. partial thickness (second degree) burn: open wound, very painful, denuded skin; blistered, moist c. full thickness (third degree) burn I. tough, leathery, dull dry, with variable pain (often severe). II. color: brown/tan/black/red: dependent on severity) III. may be life threatening; may affect many body systems d. full thickness (fourth degree) burn I. wound dull and dry II. ligaments, tendons, bone may be exposed - not painful Management: priority is to stop the burning process EMERGENCY TREATMENT OF BURNS 1. Put out fire 2. If burn is minor: a. apply cool water to burn b. remove burned clothing/jewelry c. do not disturb any blisters that form d. do not apply anything to the burn 3. If burn is serious:

a. b. c. d.

assess respiratory status and establish airway resuscitate if necessary cover burn with clean cloth arrange for transport to emergency health care facility

e. superficial: cleanse with solutions, debride loose debris or necrotic tissue, add antimicrobial ointment, and dressing (fine mesh or occlusive), tetanus prophylaxis, mild analgesic f. care of full thickness burn 1. maintain ABC: airway, breathing, circulation 2. weigh client and provide fluids and electrolytes. Use Parkland formula for burn shock 3. remove constrictive clothing or jewelry. 4. cover the burn: prevent infection, heat loss, and further deterioration 5. provide sufficient nutrition and calories to prevent negative nitrogen balance and meet body demands 6. provide adequate pain control 7. antibiotic therapy 8. tetanus prophylaxis c. care of the child with a burn wound i. aseptic technique ii. pre-medicate for pain before any dressing change iii. debridement: surgical, enzymatic, hydrotherapy iv. cleanse wounds with mild solutions, then cover with antimicrobial v. skin grafts (temporary, permanent): allograft and autografts vi. donor site, usually covered with xeroform dressing (nursing does not change this dressing) vii. long term: Jobst pressure stockings and body wraps, support surfaces, range-of-motion activities 9. Concerns in burn cases a. acute: airway status, pain, shock, infection, fluids b. long-term: nutrition, pain control, contractures, wound healing, keloid formation, psychological, body image and self esteem

X.

Pediatric Hematology Blood Components

A. Erythrocytes 1. Carry oxygen to cells and move carbon dioxide back to lungs. 2. Average life span: 120 days 3. Hemoglobin: the iron component of RBCs 4. Blood typing

a. persons with type A can receive type A or type O blood b. persons with type B can receive type B or type O blood c. persons with type O can receive only type O blood

B. Leukocytes increase with infections or inflammation:

C. Plasma proteins 1. Include albumin and globulins 2. Measuring plasma proteins helps assess nutritional status D. Platelets 1. Small fragments of cells 2. Life span eight to ten days

3. Essential to blood clotting/coagulation Disorders of Red Blood Cells E. Sickle cell anemia (SCA)

1. Definition: one of a group of diseases in which normal adult hemoglobulin (HbA) is partly or completely replaced by abnormal sickle hemoglobin (HbS) 2. Etiology: autosomal recessive disease; 1 in 2 African-Americans carries the trait, while 1 in 375 is affected with sickle cell disease 3. Pathophysiology a. trigger – RBC’s shape becomes sickled and clump b. generalized microvascular occlusion (vaso-occlusive crisis) 4. 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 exacerbations called crises: i. vaso-occlusive – painful distal ischemic usually hands and feet ii. sequestration crisis – pooling of blood in liver and spleen iii. aplastic crisis – diminished RBC production iv. hyperhemolytic crisis increased destruction of RBC 5. Diagnostics a. newborn screening (sickled ex) b. electrophoreses (hemoglobin) 6. 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 h. genetic counseling 7. Nursing interventions a. formulate teaching plan for patient and family b. encourage fluids c. apply to affected areas as ordered d. accurate I & O e. medicate for discomfort as ordered 8. Teaching points a. how to administer prophylactic antibiotics b. findings of infection c. how to increase fluid intake especially in hot weather F. B-Thalassemia 1. Definition: inherited blood disorder characterized by deficiencies in rate of production of specific globin chains in hemoglobin 2. Etiology: autosomal recessive disorder, also known as Cooley's anemia 3. Pathophysiology: abnormal, chronic production and destruction of RBC's resulting in insufficient amounts of normal circulating hemoglobin 4. Findings a. severe anemia, pallor b. microcytic RBCs c. impaired growth d. splenomegaly 5. Diagnostics a. hemoglobin and hematocrit b. hemoglobin electrophoresis c. x-rays of involved bones 6. Management a. chronic transfusion therapy to maintain hemoglobin of ten gm/dl

b. referral for chelation counseling c. genetic counseling Disorders of Excess Bleeding Summary of bleeding disorders in children:

G. Idiopathic thrombocytopenic purpura (ITP)

1. Definition: and acquired hemorrhagic disease 2. Etiology: unknown but often occurs one to two weeks after a febrile viral illness 3. Pathophysiology a. auto-immune disorder b. platelets are killed and fewer are made c. may be acute and self-limiting or chronic 4. Findings: excessive bruising, petechiae, internal bleeding 5. Diagnostics: platelet count, bleeding time, and bone marrow aspiration 6. Management: steroids, immunoglobulins (IVIG), antibody, splenectomy for chronic disease 7. Nursing interventions a. monitor for bleeding episodes b. provide for age appropriate diversional activities 8. Teaching points a. should not participate in contact sports b. should not use aspirin to relieve pain use Tylenol (acetaminophen) H. Von Willebrand's disease 1. Definition: hereditary bleeding disorder 2. Etiology: congenital (autosomal dominant) bleeding disorder; affects both males and females 3. Pathophysiology a. body makes too little von Willebrand's factor and factor VIII b. prolonged bleeding time 4. Findings: excessive bleeding of mucous membranes: gums, epistaxis (nosebleed), menorrhagia 5. Diagnostics: bone marrow aspiration 6. Management: replacement of missing clotting factor, and/or administration of antidiuretic: desmopressin acetate (DDAVP)

I.

Aplastic anemia

1. Definition: bone marrow failure 2. Etiologies a. congenital (example: Faconi's anemia) b. acquired due to exposure to overwhelming infection (hepatitis, HPV) c. causative agents such as antineoplastic agents, chemicals, chloramphenicol 3. Pathophysiology a. bone marrow stops making erythrocytes, leukocytes, and platelets (thrombocytes) b. result: pancytopenia 4. Findings: petechiae, bruising, pallor, fatigue, myelosuppression 5. Diagnostics: bone marrow aspiration 6. Therapeutic management a. antithymocyte globulin (ATG), antilymphocyte globulin (ALG) b. if severe, bone marrow transplant is treatment of choice J. Hemophilia 1. Definition: group of bleeding disorders in which there is a deficiency of one of the factors necessary for coagulation of blood 2. Etiology: x-linked recessive disorder 3. Pathophysiology: missing or defective factor VIII or factor IX blood components necessary for blood coagulation 4. Findings a. mild-severe prolonged bleeding; most often in muscles and joints (hemathrosis) b. longterm loss of range of motion of affected joints 5. Diagnostics: history of bleeding episodes, PTT, assay procedures for specific factor deficiencies 6. Management a. replacement of missing clotting factor, factor VIII concentrate b. desmopressin acetate, a diuretic (DDAVP) c. prophylactic treatment with clotting factor before surgery and some other procedures d. ice, rest, elevation, immobilization, pressure to bleeding site 7. Nursing interventions a. prevent bleeding while appropriate exercise b. brushing teeth with soft toothbrush c. recognize and control bleeding d. support family e. assess school activities 8. Teaching points a. child should wear medical identification b. genetic counseling for parents

K. Disseminated intravascular coagulation (DIC)

1. Definition: disorder of coagulation 2. Etiology a. secondary disorder of coagulation that complicates other disorders b. triggered by endothelial damage such as trauma, shock, infections, hypoxia, liver disease 3. Pathophysiology a. the first stage of the coagulation process is abnormally stimulated b. clotting mechanism is triggered in circulation, thrombin is generated in greater amounts than the body can neutralize c. rapid conversion of fibrinogen to fibrin with aggregation and destruction of platelets d. local and widespread fibrin deposition in blood vessels causes obstruction and necrosis e. fibrinolytic mechanism causes extensive destruction of clotting factors 4. Findings a. bleeding, bruising, petechiae b. altered serum levels of clotting-related factors (increased PT, PTT, TT, decreased platelets, degraded fibrinogen) c. clotting, hypoxemia, intracranial hemorrhage, progressive organ failure 5. Diagnostics: PT, PTT and TT (Thrombin time) 6. Management a. control of underlying etiology b. factor replacement c. platelets, fresh frozen plasma (FFP) and RBC transfusions d. vitamin K e. oxygen 7. Nursing Interventions a. monitor IV infusions, blood transfusions b. administer Heparin as ordered c. monitor any severely ill child to recognize presence of this disorder XI. Pediatric Oncology I. Cardinal findings of cancer in children I. Unusual mass II. Pallor III. Sudden tendency to bruise IV. Rapid, unexplained weight loss V. Change in vision or eye

II.

VI. Recurrent fever VII. Persistent headache, often with vomiting VIII. Change in balance or gait Leukemias - most common cancer in children

I. II.

III.

IV. V.

VI.

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 III. lymphadenopathy, hepatosplenomegaly, bone or joint pain IV. meningeal irritation – irritable, lethargic, stiff neck Diagnostics: history, physical findings, peripheral blood smear, and bone marrow aspiration Management I. combination chemotherapy to achieve a remission II. radiation if CNS involved or intrathecal administration of methotrexate III. bone marrow transplant Nursing interventions I. prepare child and family for diagnostic procedures II. relieve discomfort III. monitor for infection, hemorrhage and anemia IV. manage problems of drug side effects – nausea and vomiting, anorexia, mucosal ulceration, neuropathy, alopecia and moon face.

VII.

V. provide nutritional snacks VI. refer for needed services – financial or home care Teaching points I. long term treatment plan II. multidisciplinary approach III. encourage parents to ask questions when unsure IV. school work for child when appropriate

Lymphomas

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)

3. Findings a. characterized by painless enlargement of lymph nodes, particularly in supraclavicular area b. anorexia, weight loss, malaise; painless; night sweats c. fever 4. Diagnostics: lymphangiography, CBC, Sed Rate, urinalysis, CT Scan, chest x-ray 5. Management a. chemotherapy and radiation b. treatment based on staging of disease (stages I-IV) 6. Nursing Interventions a. preparation for diagnostic procedures b. explain side effects of treatment c. provide age appropriate diversional activities d. refer to appropriate resources if sterility may result from irradiation and/or chemotherapy 7. Teaching points a. long term treatment plan b. multidisciplinary approach c. encourage to ask questions when unsure d. school work for child when appropriate D. Non-Hodgkin's lymphoma

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 E. Brain tumors: medulloblastoma, astrocytoma, ependymoma, glioblastoma 1. Pathophysiology a. solid tumors of the nervous system b. most common solid tumor in children c. location extremely important 2. Findings a. headache, vomiting, increased intracranial pressure, ataxia b. visual changes, seizures, bulging fontanel in infants, behavioral changes 3. Diagnostics: history, MRI, CT Scan, EEG, and lumbar puncture 4. Management a. surgical debulking b. radiation c. chemotherapy 5. Nursing interventions a. neuro signs b. vital signs c. prepare child and family for diagnostic and operative procedures – possible shaving of head d. positioning considerations depend on procedure done e. monitor dressing and ICP, if appropriate f. begin feeding when ordered g. medicate for discomfort as indicated 6. Teaching points a. child’s post operative appearance b. refer for needed resources F. Neuroblastoma 1. Pathophysiology a. solid tumor found only in children under four years-old b. primary sites are adrenal gland or retroperitoneal because it starts from embryonic neural crest cells of the adrenal gland and sympathetic nervous system. c. often has metastasized by the time it is diagnosed 2. Findings a. firm, nontender mass in the abdomen that crosses the midline b. distant metastasis may cause periorbital edema (swelling around eyes) 3. Therapeutic management a. treatment is dependent on staging criteria b. surgical debulking, chemotherapy, radiation G. Other tumors

Summary overview of other tumors in children:

H. Osteosarcoma 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. Diagnostics: history, physical assessment, chest x-ray, MRI, CT scans, and radioisotope bone scans 4. Therapeutic management: surgical approach a. limb salvage with prosthetic bone replacement; or amputation b. plus combination chemotherapy before and/or after surgery I. Ewing sarcoma 1. Pathophysiology a. arises not from osteoid tissue but in bone marrow spaces b. most common in shaft of femur, tibia, humerus, scapula c. more common in males 2. Findings: localized pain, decrease in physical activity 3. Therapeutic management a. intensive radiation therapy of the malignant bone b. combined with chemotherapy c. surgery not routinely recommended J. Rhabdomyosarcoma 1. Pathophysiology a. soft tissue neoplasm b. grows from undifferentiated mesenchymal cells of skeletal muscle c. most common in head and neck, especially the orbit d. staging I-IV e. highly malignant; often metastasized when diagnosed 2. Findings a. non-tender, firm mass b. related to site of tumor and compression of adjacent organs c. symptoms often vague, similar to otitis media or "runny nose" 3. Therapeutic management a. high-dose irradiation of the primary tumor b. combination chemotherapy

c. surgical resection K. 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. Diagnostics: ophthalmoscopic exam under general anesthesia, ultrasound and CT scan 4. Management a. radiation therapy, enucleation, chemotherapy b. treatment depends on stage of tumor, stages I-V 5. Nursing interventions a. assess child for this when parents report a strange light in the child’s eye b. involve parents in care and teach about diagnostic procedures 6. Teaching points a. demonstrate positive aspects of prosthesis – show pictures of other affected children after surgery b. genetic counseling for parents 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 anticonvulsants 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 the 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. 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. 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. The 3 C's - cough, choke, and cyanosis. 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. Pulse - distal to the facture 3. Pallor 4. 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. Children's soft tissues are 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.

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