Disease Atrial septal defect (ASD) s/s Small asymptomatic Large poor growth, fatigue, CHF Systolic murmur Patho Opening between R and L atria AV canal (AVSD) First months of life HF Loud systolic murmur Cyanosis that w/ crying Patent ducturs arteriosis (PDA) Holosystolic murmur Low ASD High VSD Cleft deformity of tricuspid and mitral valves Persistent channel between pulmonary artery and aorta after birth Aortic stenosis: newborn Faint pulses Hypotension Tachycardia Poor PO feeding (s/s from CO) Exercise intolerance CP Dizziness when standing for long period Systolic ejection murmur (sometimes) Loud systolic ejection murmur Aortic stenosis: children Pulmonic stenosis Coarctation of the aorta High BP (in arms, lower BP in legs Bounding pulses in arms Weak/absent femoral pulses Cool lower extremities Infants HF, Rapid deterioration of hemodynamic stability Narrowing of aortic valve resistance from BF to LV CO LV hypertrophy HF Narrowing of the opening of pulmonary artery or pulmonary valve itself Narrowing near the portion of aorta where the ductus arteriosus inserts causes: 1. pressure to head and upper extremities 2. pressure to body and lower extremities Nursing stuff Must be NPO prior to indomethacin administration Tx Spontaneous closure (within 4 yrs) Surgical patch (if not closed within 2 yrs + CHF s/s) Open heart Sx (usually complete repair) patch ASD/VSD Reconstruction of valves Other important info Indomethacin ( risk for necrotizing enterocolitis) Sx RF = O2 administration after birth, prematurity Most common CHD in Down’s syndrome Balloon dilation in cath lab (1st line) Stage 1 of Norwood procedure (if critical) Aortic vavotomy (palliative procedure) Balloon dilation of valve surgical valvotomy Surgical resection < 6 mo sx > 6 mo balloon angioplasty Restenosis can happen risk for HTN, ruptured aorta, aortic aneurysm, stroke Tetralogy of Fallot (TOF) Older kids dizziness, HA, fainting, epistaxis from HTN Cyanosis Systolic murmur of moderate intensity Acute episodes of hypoxia or cyanosis (TET spells or blue spells) occurs when O2 supply exceed oxygen supply in the blood - Usually when feeding or crying - At risk for PE, seizures, LOC, sudden death after anoxic spell - Nursing intervention squat Tricuspid atresia cyanosis tachycardia dyspnea clubbing (older kids) Transposition of the great arteries (TGA) Cyanosis HF Murmur Cardiomegaly (within wks of birth) 4 Defects: VSD Pulmonic stenosis R ventricular hypertrophy Overriding aorta tricuspid valve doesn’t develop no blood flow from RA to RV blood flow is dependent on: - R side to L side of heart ASD or PFO - Pulmonary artery to lungs PDA - L side to R side of heart VSD Pulmonary artery leaves the LV Aorta leaves RV PFO can be present (but not always) most common defect associated w/ TGA When pt crying or feeding and has hypoxic episode, have pt squat surgical Until sx, prostaglandins given to keep PDA open Sx = modified fontan procedure Lab monitoring (metabolic acidosis, polycythemia, anemia) Therapeutic: prostaglandins to keep PDA open Rashkind procedure Sx Rastelli procedure Truncus arteriosus HF Cyanosis Poor growth Activity intolerance Hypoplastic left heart syndrome (HLHS) Mild cyanosis HF When PDA closes, cardiac collapse Acquired Heart Disease Infective Endocarditis fever Roth spots Osler nodes murmur Janeway lesions Anemia Nail-bed hemorrhages Emboli VSD can be present (but not always) will increase likelihood of HF Embryonic structures fail to separate into a pulmonary artery and an aorta and combine to form one great vessel (i.e. truncus) 3 types: - R & L PA arise separately from truncus - R & L PA arise separately from truncus but are in close proximity to truncus - R & L PA arise from truncus independently Underdevelopment of L side of heart leading to CO - Hypoplastic L ventricle - Aortic atresia ASD & PFO & PDA - Allows blood to flow to the body Inflammation from an infection of the cardiac valves and endocardium Usually occurs in conjunction w/ another form of heart disease Pressure gradients cause turbulent blood flow leading to blood clots Lab monitoring (metabolic acidosis, polycythemia, anemia) Sx repair in 1st month of life Lab monitoring (metabolic acidosis, polycythemia, anemia) Norwood procedure Glenn shunt procedure Modified Fontan procedure heart transplant Assess for ABX s/e Ed importance of f/u, infection control prophylactic ABX before dental procedures High-dose ABX 2-8 wks f/u testing Echo, blood cultures sx valve repair if needed prophylactic ABX before dental procedures Rheumatic fever fever chorea joint pain carditis nodules erythema marginatum Kawasaki disease fever > 102.2F strawberry tongue red, cracked lips cervical lymphadenopathy red soles and palms conjunctival redness lethargy, irritability cardiac complications coronary artery aneurysm intermittent colicky abdominal pain rash over trunk and perineal area ECG changes Slow/fast HR Poor perfusion s/s Dizziness, syncope Poor feeding Dysrhythmias (fibrin and platelets) that can entrap microorganisms Vegetations =the structures formed by this process inflammatory condition of connective tissue abnormal humoral and cell-mediated immunologic response typically 2-6 wks after untreated GABHS infection in pharynx inflammation is caused in brain, heart, skin joints bed rest initially and reduced activity until symptoms subside primary prevention = prompt Dx + ABX (usually PCN) pt ed no live vaccines for 11 months after IVIG monitor for fever IVIG “ + high-dose ASA therapy Glucocorticoids (maybe) assessment, early identification, quick response is critical don’t leave pt note when rhythm starts and how long it lasts count apical pulse for FULL minute notify HCP Vagal maneuver (for SVT) Cardioversion Meds CPR Hypertrophic Cardiomyopathy Allergic rhinitis Laryngomalacia Croup Epiglottitis Normal PE Adolescents - Palpitations - Syncope - Dyspnea - Pain w/ exertion - Systolic murmur L sternal border or apex (louder as pt goes from laying down to standing) Infants and children fatigue easily Rhinorrhea itching Paroxysmal sneezing Allergic salute Allergic shiners Noisy, crowing respiratory sounds w/ or w/o retractions in neonatal period sudden onset of harsh, metallic, barky cough sore throat inspiratory stridor hoarseness use of accessory muscle to breathe agitation cyanosis drooling dysphagia dysphonia distressed respiratory efforts Pt ed no strenuous activity or contact sports, hydration, med Be prepared for emergencies Congenital laryngeal stridor Immature neuromuscular development of the airway usually caused by parainfluenza virus H. influenzae No strenuous activity or contact sports!! If asymptomatic beta blocker or Ca channel blocker Symptomatic septal myomectomy, pacemaker s/s resolve by 18-24 months do not leave child unattended obtain culture but be careful not to stimulate anything (it could cause airway obstruction) respiratory syncytial virus (RSV) = cause of 50% of cases Bronchiolitis Pneumonia (viral) Pneumonia (bacterial) Bronchopulmonary dysplasia (Hyaline membrane disease) Acute otitis media Otitis media with effusion Viral pharyngitis high fever cough crackles Preceded by URI Abrupt onset High fever Cough CP s/s COPD (aka poor perfusion) Otalgia (earache) may pull ear(s) or roll head Bulging, red, opaque tympanic membrane Diffuse tympanic light reflex Yellowish-green, purulent, foul-smelling drainage indicates perforation of tympanic membrane Tinnitus w/ popping sounds Conductive hearing loss below 35 decibels Mild balance disturbances Gradual onset w/ sore throat Erythema (inflammation) of pharynx and tonsils Vesicles/ulcers on tonsils Fever (usually low-grade but may be high) influenza RSV Group A strep Mycoplasma Chlamydia Trachomatis Thickening of alveolar walls and bronchiolar epithelium Caused by mechanical ventilation usually highly communicable contact isolation Supportive O2 Fluid therapy ABX O2 Fluid therapy Chest tube drainage if needed significant cause of hospitalization in children under 1 year common in winter and spring (“RSV season”) Most common < 3 yrs Occurs mostly in low birth weight and premature infants Lasts 3-4 days Bacterial pharyngitis Hoarseness, cough, rhinitis, conjunctivitis, malaise, anorexia (early) Maybe cervical lymph node enlargement/tenderness Abrupt onset Severe sore throat Erythema of pharynx and tonsils High fever Abdominal pain, HA, vomiting Maybe cervical lymph node enlargement/tenderness Lasts 3-5 days Nebulizer use asthma Cystic Fibrosis Prune Belly Syndrome Thick, tenacious mucus plugs Obstruction and dysfunction of pancreas, lungs, salivary glands, sweat glands, and reproductive organs Small, frail appearance (bc pancreatic enzymes can’t break down nutrients) Barrel chest Protuberant abdomen Clubbing Absence of abdominal muscles Vesicoureteral reflux (VUR) Inherited multisystem disorder Characterized by widespread dysfunction of exocrine glands Autosomal recessive trait Mechanical postural mucus drainage Chest PT teaching Diet high protein, high fat, caloric intake bronchodilators ABX Pancreatic enzymes (give w/ each meal) Monitor for UTIs Suprapubic catheter/vesicostomy Oxchiopexy abdominoplaty Asthma action plan tells what meds to use based on s/s and respiratory status (give to school, sports coach, etc.) Acute Post-streptococcal glomerulonephritis Nephrotic syndrome Bladder Extrophy Hydroureteronephrosis Bilateral cryptorchidism (undescended testicle) Megalourethra 30% develop ESRD hematuria proteinuria HTN Edema Renal insufficiency Edema Anorexia Fatigue Abdominal pain weight Respiratory infection Elevated serum cholesterol and triglycerides **proteinuria Bladder is open and outside pelvis Males penile tube is not closed either Causes hypercoaguable state risk for thrombus Anal irritation Social withdrawal Surgery to close bladder and put into pelvis Full neural tube closure doesn’t occur during fetal development Esophageal atresia = esophagus ends before the stomach TE fistula = fistula (unnatural connection) forms between esophagus and trachea stomach content can enter lungs, air can enter stomach Esophageal Atresia w/ TE Fistula Encopresis Immune response to strep virus products of inflammatory response damage glomerular capillaries GFR renal insufficiency Damage to capillary walls permeability hematuria/proteinuria Risk for aspiration! Assess respiratory status! Surgery to remove fistula and attach esophagus to stomach Tx = constipation Tx 50% of cases have additional anomalies will likely get many tests Ulcers Urinary incontinence/UTI Soiled clothing *repeated, involuntary defecation in kids > 4 yrs who have normal colon and rectal anatomy Pain, Burning Cramping on empty stomach Waking up w/ stomach pains < 6 yrs vomiting Usually caused by H.pylori Replace water Correct acid-base Infectious gastroenteritis Appendicitis Rebound pain at McBurney point GERD Spit up Baby uncomfortable (arches back, cries) Usually worse at night and after meals Sometimes resp. s/s cough, stridor Ulcerative Colitis Crohn’s Disease Hypertrophic Pyloric Stenosis Diarrhea Malabsorption May be smaller than average kid their age Especially for Crohn’s growth, sexual maturation > 3 wks old Progressive projectile vomiting (nonbilious) Movable and palpable olive-shaped mass See pg. 977 (review table) Positioning for pain control = knee to chest position! Pt ed small frequent meals, positioning *only affects the colon *only affects mucosal and submucosal layers of GI tract Diet ed avoid milk products, low fiber, low fat, high protein, low residue, hypoallergenic diet *can affect any part of digestive tract (from mouth to anus) *can affect all layers of GI tract Complications = dehydration, metabolic acidosis Meds immunosuppressants, etc. Dietary modification Psych help (d/t peer isolation from frequent hospitalization, etc.) Sx to release obstruction Can be cured w/ removal of colon Lifelong condition Intussusception Weight loss Sudden abdominal pain Palpable mass Abdominal distention Currant jelly stools Volvulus Pain Bilious vomiting s/s bowel obstruction Hirshspurng Disease Bowel obstruction s/s (distended abdomen, bilious vomiting, feeding intolerance, delay in passage of meconium after birth Lactose intolerance Celiac disease Short bowel syndrome Viral Hepatitis s/s celiac crisis profuse, watery diarrhea and vomiting chronic growth deficiency, folate deficiency, irritability, diarrhea Watery diarrhea Malabsorption s/s (bloating, foul-smelling stool, excessive gas, etc.) Type A little or no s/s (maybe fatigue, anorexia) Type B can range from asymptomatic to fatal Telescoped portion of bowel causing obstruction Malrotation/twisting of bowel causes bowel obstruction (usually defect in fetal development) Absence of ganglion cells in colo-rectal area Watch for LOC, fever, HR, respiratory distress, BP changes may indicate sepsis/shock/peritonitis (report ASAP) Stable hydrostatic reduction Unstable or if reduction fails surgery Emergency! Treat within 12 hrs Surgery Ed colostomy care (if needed), rectal irrigation education and show how, hydration Surgically remove aganglionic segments of bowel Absence/deficiency of lactase AA Dietary intervention Not enough gut Nutritional support = key Maybe intestinal transplant Avoidance of gluten Highly contagious Vaccination for Hep A & B Let them know it may get worse before it gets better (so they don’t freak out if baby turns jaundiced) Dx = biopsy to assess for missing ganglionic cells Celiac crisis = life threatening Viral is most common in children Type B can be spread perinatally No OTC meds during infection Eosinophilic Esophagitis (EOE) Dysphagia Abdominal pain Vomiting Feeding impaction Feeding dysfunction Necrotizing Entercolitis Early s/s abdomen enlargement/tenderness, bilious vomiting Developmental delays common Cystic fibrosis Abnormal secretions of thick, tenacious mucus Obstruction/dysfunction of pancreas, lungs, salivary glands, sweat glands, reproductive organs Positive kernig sign Positive brudiznski sign Splotchy, red, petechial rash on trunk (red flag sign) Vary depending on cause and level of irritation Autosomal recessive genetic disorder characterized by widespread dysfunction of exocrine glands Café-au-lait spots Multiple neurofibromas and benign tumors grow under skin beginning in puberty Autosomal dominant genetic disorder Tumors grow along nerves Meningitis (bacterial) Encephalitis Neurofibromatosis Large number of eosinophils collect in the esophagus causing injury and irritation Usually allergic response to food Walls of intestine become damaged and can perforate allowing bacteria and fecal matter to leak Inflammation of meninges Elimination diet PPIs Steroids PO Watch the belly carefully! measure abdominal circumference Ed long-term f/u w/ GI needed ABX + surgery Most common GI emergency in NICU Babies at most risk = VLBW, <32 wks (90% of cases) Breastfed babies at lower risk Bronchodilators Respiratory treatments ABX Pancreatic enzymes NPO Monitor for ICP Prevention = vaccination! Risk for seizures! seizure precautions Monitor cardiorespiratory function Careful eye exams assess growth and development ABX Dexamethasone Viral meningitis is more common and has less severe symptoms; resolves spontaneously in 3-10 days Dx: Hx + exposure Hx (travel, insect bites) Labs CSF cultures Nasal swabs Stool specimens CT/MRI/EEG Hydrocephalus learning disabilities and hyperactivity s/s vary with age and degree Reye Syndrome Guillain-Barré Syndrome Cerebral Palsy (CP) Imbalance between production and absorption of CSF Acute encephalopathy caused by toxic, inflammatory, or anoxic insult/injury Rapid onset of symmetric motor weakness Progresses in ascending pattern Areflexia High protein in CSF Acute muscle denervation (shown w/ electroconduction tests) Variable levels of intelligence and functional capacity Developmental delay Abnormal motor performance, abnormal DTRs Altered muscle tone (central hypotonia, hypertonia in extremities) Monitor head circumference, s/s infection, LOC changes, appetite, sleep patterns Monitor abdominal circumference (for internal shunts) Careful positioning (flat) Slowly raise HOB w/ internal shunt over course of a few days Monitor output DO NOT MOVE!! **no ASA use in kids! Acute, inflammatory, demyelinating polyneuropathy AA triggered by infection Pain management = key Monitor resp. status Caused by nonprogressive abnormality or injury to developing brain H&P (especially developmental milestones Hx, neuro, musculoskeletal) Shunt, remove obstruction, divert excess CSF PICU admission for supportive care and prevention of secondary complications IVIG or plasmapheresis Goal = optimize abilities within confines of dysfunction Promote socialization Self-help w/ ADLs PT/OT/SLP Behavioral therapy IEP/504 Clinical classifications: Spastic CP (most common) Dyskinetic CP Ataxic CP Mixed type Often not diagnosed until 6-12 months old Abnormal posture Sensory deficits Speech difficulties Seizures Dental caries Down Syndrome (Trisomy 21) Fragile X syndrome Developmental hip dysplasia Talipes Equinovarus (clubfoot) Simlan crease on palms Intelligence varies (most have moderate intellectual impairment) Delayed development (potty training takes longer, etc.) Men = infertile Long face w/ prominent jaw Large protruding ears High palate Enlarged testicles Cognitive impairment maybe (80% in boys, 30% in girls) Speech/language delays Easily overstimulated Shortened limb on affected side Restricted abduction of hip on affected side Asymmetric thigh and gluteal folds Positive Ortolani and Barlow tests Children abnormalities of gait (limping, toewalking, waddling gait) **need discipline based on developmental age, NOT chronological age Watch for feeding difficulties Monitor speech & language development Antispasmodic meds baclofen (PO or pump), botox Anxiety and depression interventions PRN PT/OT/SLP Crucial to ID and start therapies early to avoid disintegration of intelligence Newborns Pavlik harness (maintains hips in flexion, abduction and external rotation) After infancy surgery, spica casting Congenital dysplasia of all tissue below the knee Pondryi casting + French physiotherapy Complications = contractures, hip dislocation (in nonambulatory kids) Most common genetic birth defect RF = mom > 35 yrs, +FHx Legg-Calve’-Perthes Disease Scoliosis Slipped Capital Femoral Epiphysis (SCFE) Osteogenesis Imperfecta (Brittle Bone Disease) Juvenile Idiopathic Arthritis Neurofibromatosis Usually in kids who are shorter than average and LBW infants Persistent pain of the hip that worsens w/ movement Limited ROM Can be intermittent Shoulder/hip/skin fold asymmetry Unequal scapular prominence Usually in overweight adolescent boys Limp and pain Pain to groin, hip, thigh or knee intermittent and worsens w/ activity Externally rotated on presentation Thin/translucent skin Short stature Blue sclera Shepherd crook deformity Swelling in at least 1 joint for at least 6 wks WITH 2+ signs of ROM, discomfort w/ movement or increased warmth Café-au-lait spots Multiple neurofibromas and benign tumors grow under skin beginning in puberty learning disabilities and hyperactivity Management no weight bearing! Aseptic necrosis of femoral head Lateral curvature of spine (> 10 degrees) *Adams forward bend test tests for spinal abnormalities (used for screenings) Shearing stress causes femoral head to displace from its normal position relative to the femoral neck Immediate bed rest Internal fixation Weight control and dietitian referral Defect of collagen synthesis Fracture w/ minimal stress No cure Symptom management Chronic AA Starts before 16 yrs Chronic inflammation of synovium w/ effusion leading to destruction of articular cartilage Autosomal dominant genetic disorder Tumors grow along nerves PT/OT Meds NSAIDs, DMARDs, biologics Careful eye exams assess growth and development Bacterial pharyngitis Abrupt onset (may be gradual in children < 2 yrs) Sore throat (usually severe) Erythema, inflammation of pharynx and tonsils High fever Abdominal pain, HA, vomiting Cervical lymph nodes may be enlarged, tender Lasts 3-5 days Viral pharyngitis Gradual onset w/ sore throat Erythema, inflammation of pharynx and tonsils Vesicles or ulcers on tonsils Fever (usually low-grade but can be high) Hoarseness, cough, rhinitis, conjunctivitis, malaise, anorexia (early) Cervical lymph nodes may be enlarged, tender Lasts 3-4 days
