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178 Cards in this Set
- Front
- Back
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Top Childhood Cancers -
What are they |
1. Leukemia (ALL)
2. CNS tumors 3. Lymphoma 4. Neuroblastoma |
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Neuroblastoma -
What is it Associated with? |
Embryonal tumor of
neural crest cell origin MC cancer in infants more than 1/2 kids < 2 y/o associations - neurofibromatosis Hirschsprung's n-myc oncogene |
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Neuroblastoma -
Hx/PE |
Can occur anywhere
Sx vary with location nontender abdom mass (may cross midline) Horner's syndrome HTN cord compression anemia FTT fever site-specific mets can cause- proptosis periorbital bruising subq tumor nodules bone pain with pancytopenia opsoclonus/myoclonus "dancing eyes, dancing feet" |
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Neuroblastoma -
Dx |
Abdom CT
24-hr urinary catecholamines assess extent of disease - CXR bone scan CBC LFTs BUN/Cr coag panel |
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Neuroblastoma -
Tx |
■ Excision - localized tumors
■ chemo includes - cyclophosphamide doxorubicin ■ adjunctive radiation - if tumor spread beyond origin ■ prognosis improved for kids with low-risk dis. - < 1 y/o no N-myc amplification localized |
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Wilms' Tumor -
What is it Associated with? |
Embryonal cancer of kidney
MC renal tumor in kids 2-4 y/o associated with - family Hx Beckwith-Wiedemann syndrome WAGR neurofibromatosis |
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Wilms' Tumor -
Hx/PE |
Painless, palpable abdom mass
does not cross midline n/v fever weight loss hematuria HTN |
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Wilms' Tumor -
Dx |
Abdom CT or US -
intrarenal mass check for metas - CXR chest CT CBC LFTs BUN/Cr |
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Wilms' Tumor -
Tx |
■ Transabdom nephrectomy
■ postsurgical chemo - vincristine dactinomycin ■ flank irradiation (for some) ■ prognosis good - depends on staging & histo |
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RDS -
What is it |
MCC of resp failure
in premies surfactant deficiency => inc. surface tension (poor lung compliance) and alveolar collapse (atelectasis) surfactant made by T2 pneumocytes mainly ~35th week dipalmitoyl phospha- tidylcholine risk factors - maternal diabetes males 2nd born of twins |
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RDS -
Hx/PE |
Presents in 48-72 hrs of life
RR > 60/min intercostal retractions expiratory grunting nasal flaring cyanosis progressive hypoxemia |
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RDS -
Dx |
ABGs
CBC (to r/o infection) BC (to r/o infection) CXR - bilat diffuse atelectasis causing ground-glass appearance with visible air bronchograms lecithin:sphingomyelin ratio < 2 |
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RDS -
Tx |
CPAP or
intubation and mech vent artificial surfactant to prevent - mom gets corticosteroids monitor fetal lung maturity (L:S ratio) |
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RDS -
Complications |
Persistent PDA
bronchopulmonary dysplasia retinopathy of prematurity intraventricular hemorrhage NEC |
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Intussusception -
What is it |
MCC of bowel obstruction in
1st 2 yrs. of life males > females usu prox to ileocecal valve cause - idiopathic in older kids - mass or intest. abnormality triggers the telescoping: adenovirus or rotavirus parasites CF celiac disease polyps intestinal lymphoma Meckel's diverticulum Henoch-Schonlein purpura |
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Intussusception -
Hx/PE |
Hx -
colicky abdom pain in apparently healthy kids n/v too young to talk - cry draw knees up to chest dyspnea with pain advanced signs - red "current jelly" stool lethargy fever PE - abdom tenderness pos. stool guaiac palpable "sausage-shaped" RUQ abdom mass |
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Intussusception -
Dx |
Abdom XR
abdom US air contrast barium enema CBC |
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Intussusception -
Tx |
Correct vol & electrolytes
check CBC air-contrast barium enema - diagnostic and therapeutic surgical reduction or resection (if gangrenous) |
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Pyloric Stenosis -
What is it |
Hypertrophy of
pyloric sphincter 1st-born males more affected |
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Pyloric Stenosis -
Hx/PE |
1st 2 wks - 4 mos. of life
nonbilious emesis => projectile emesis after each feeding so, babies feed well init => malnutrition & dehydration palpable olive-shaped, mobile, NT epigastric mass visible gastric peristalsis |
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Pyloric Stenosis -
Dx |
Abdom US - diagnostic
barium studies - string sign pyloric beak hypochloremic, hypokalemic metabolic alkalosis |
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Pyloric Stenosis -
Tx |
First -
hydration correct acid-base & electrolyte abnormalities NG tube - possible long. pyloromyotomy |
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Child Abuse -
What is it |
Neglect
physical abuse sexual abuse emotional abuse suspect - if Hx doesn't match physical findings if there was a delay in getting medical care |
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Child Abuse -
Hx/PE |
Infants may have apnea,
seizures, FTT Exam findings include - . cutaneous - ecchymoses of varying ages patterned injuries . skeletal - . spiral fractures of femur and humerus in kids < 3 = abuse unless prove else . epiphyseal/metaphyseal injuries - can happen in infants from pulling/twisting limbs . rib injuries < 2 y/o . sexual abuse - STDs or genital trauma |
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Child Abuse -
Dx |
R/o conditions that mimic
skeletal survey & bone scan - can show fractures in various stages of healing if sexual abuse suspected - test for gonorrhea, chlamydia and HIV to r/o shaken baby syn - check for retinal hemorrhages CT for subdural hemorrhages MRI for white matter changes |
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Child Abuse -
Tx |
Document injuries
notify child protective svcs hospitalize if nec. |
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Epiglottitis -
What is it |
Serious, rapidly progressive
infection of supraglottic before immunization - from H influ type B now - Streptococcus nontypable H flu viral agents |
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Epiglottitis -
Hx/PE |
Sudden-onset high fever
dysphagia drooling muffled voice soft stridor cyanosis "sniffing dog" position "tripod" position insist on sitting up in bed untreated - life-threatening |
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Epiglottitis -
Dx |
Clinical
DON'T EXAMINE THROAT unless anesthesiologist present definitive Dx - direct fiberoptic visual of cherry-red, swollen epiglottis & arytenoids lat XR - thumbprint sign |
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Epiglottitis -
Tx |
Emergency
call anesthesiologist transfer pt. to OR endotrach intubation or trach IV ABx - ceftriaxone or cefuroxime |
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Croup (Laryngotracheobronchitis) -
What is it |
Inflammation of larynx and
upper airway, mainly subglottic space => narrowing of airway kids 3 mos. - 3 yrs. MCC - parainfluenza virus 1 also - PIV-2, PIV-3, RSV, rubeola, influenza, adenovirus, Mycoplasma pneumonia |
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Croup (Laryngotracheobronchitis) -
Hx/PE |
Prodrome - URI Sxs 1-7 days
stridor - worse by agitation fever - low grade hoarseness barking cough |
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Croup (Laryngotracheobronchitis) -
Dx |
Clinical
XR - steeple sign |
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Croup (Laryngotracheobronchitis) -
Tx |
Mild - cool mist
moderate - oral corticosteroids severe - (resp. distress at rest) admit nebulized racemic epi |
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Bronchiolitis -
What is it |
Acute inflammation of
smallest airways acute viral bronchiolitis MCC - RSV infants & kids < 2 y/o can progress to resp. failure risk for severe RSV - < 6 mos. old premies heart or lung dis. immunodeficiency |
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Bronchiolitis -
Hx/PE |
Hx -
low-grade fever rhinorrhea cough apnea - young infants PE - tachypnea wheezing hyperresonance to percussion |
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Bronchiolitis -
Dx |
CXR - hyperinflation of lungs
interstitial infiltrates atelectasis ELISA of nasal washings for RSV - hi sens & spec |
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Bronchiolitis -
Tx |
Mild -
outpt. fluids, nebulizers, O2 if nd admit if - marked resp distress O2 saturation < 95% toxic appearance dehydration/poor oral feeding premie (< 34 wks) < 3 mos. old underlying cardiopulmon dis. unreliable parents inpatients - contact isolation hydration O2 ribavirin RSV prophylaxis - RespiGam or Synagis high-risk pts. in winter |
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Otitis Media -
What is it Risk Factors |
Middle ear infection
MCC - #1 - S. pneumoniae #2 - H. flu #3 - Moraxella catarrhalis kids predisposed - eusta tube risk factors - viral URIs trisomy 21 CF immunodeficiency smoke exposure day-care attendance bottle feeding cleft palate prior otitis media |
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Otitis Media -
Hx/PE |
Fever
ear tugging hearing loss irritability Erythema bulging decreased mobility of tym memb loss of light reflex and bony landmarks tym memb may be perforated |
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Otitis Media -
Dx |
Clinical
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Otitis Media -
Tx |
Amoxicillin - 10 days
Tx failure after 3 days - switch to amoxicillin-clavulanic acid, ceftriaxone or cefuroxime |
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Otitis Media -
Complications |
Mastoiditis
meningitis hearing loss cholesteatoma tympanosclerosis chronic suppurative OM |
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Kawasaki Disease
(Mucocutaneous Lymph Node Syndrome) - What is it |
Multisystem acute vasculitis
usually kids < 5 y/o esp. Asian at risk for coronary artery aneurysms => MI |
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Kawasaki Disease
(Mucocutaneous Lymph Node Syndrome) - Hx/PE |
"CRASH and Burn"
Conjunctivitis - b/l Rash Adenopathy - cervical lymph Strawberry tongue Hands and feet - swollen, red, desquamation fever > 40C for > 5 days |
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Kawasaki Disease
(Mucocutaneous Lymph Node Syndrome) - Dx |
Clinical
thrombocytosis - wk 2 or 3 inc. ESR |
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Kawasaki Disease
(Mucocutaneous Lymph Node Syndrome) - Tx |
High-dose aspirin
IVIG - to prevent aneurysms corticosteroids are contraindicated (they may inc. aneurysms) |
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FTT -
What is it |
Persistent weight
below 3rd to 5th percentile or falling off growth curve organic - medical condition nonorganic - psychosocial nonorganic is MC risk factors - chronic illness poverty low maternal age chaotic envi genetic dis. (CF) inborn errors of metabolism HIV |
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FTT -
Hx/PE |
Low weight for age and height
minimal weight gain or weight loss plot on growth chart ck for signs of systemic dis. diet Hx observe caregiver-child interaction |
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FTT -
Dx |
Calorie count
CBC electrolytes Cr albumin total protein sweat chloride test UA/UC stool culture O&P assess bone age |
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FTT -
Tx |
Tx depends on cause
supplement nutrition if breastfeeding inadequate admit if - neglect severe malnourishment |
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Atrial Septal Defect (ASD) -
What is it |
Opening in atrial septum
lets blood flow bet. atria L to R shunting due to lower R pressure blood flow to lungs inc. |
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Atrial Septal Defect (ASD) -
Hx/PE |
Hx -
usu. presents in late childhd or early adult onset & severity dep. on size large defect - tire easy (DOE) freq. resp. infections FTT => CHF => cyanosis PE - RV heave wide, fixed split S2 systolic ejection murmur - upper left sternal border |
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Atrial Septal Defect (ASD) -
Dx |
Echo with color flow Doppler -
diagnostic ECG - rt-axis deviation CXR - cardiomegaly and inc. pul vascular markings |
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Atrial Septal Defect (ASD) -
Tx |
. Small defects may close
spontan (no Tx needed) . ABx prophylaxis before dental procedures . surgical closure - infants with CHF pts. > 2:1 pul to sys bld flow correct early to prevent - arrhythmia RV dysfunction Eisenmenger's syn |
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Ventric Septal Defect (VSD) -
What is it |
MC congenital heart defect
More common in pts. with - Apert's syn Cri-du-chat Trisomies 13 & 18 |
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Ventric Septal Defect (VSD) -
Hx/PE |
Hx -
Sxs dep. on degree of shunting small def usu asymp at birth large def - CHF freq resp infections FTT PE - pansystolic murmur - lower lt sternal border loud pulmonic S2 in severe defects - systolic thrill cardiomegaly crackles |
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Ventric Septal Defect (VSD) -
Dx |
Echocardiogram - diagnostic
EKG - may show RVH or LVH normal with small VSDs |
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Ventric Septal Defect (VSD) -
Tx |
. Most small defects close
spontan (no Tx needed) . ABx prophylaxis before dental or pulmonary procedures . surgical closure - correct early to prevent - Eisenmenger's syn, et al . Tx CHF & resp infections |
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Patent Ductus Arteriosus (PDA)
What is it |
Failure of DA to close
=> L-to-R shunt (aorta to pulmonary artery) risk factors - high altitude (low O2) 1st trimester rubella in mom premies females (more common) |
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Patent Ductus Arteriosus (PDA)
Hx/PE |
Hx -
typically asymp (small PDA) slowed growth recurrent lwr resp infections lwr extremity clubbing CHF Sxs PE - wide pulse pressure continuous machine murmur loud S2 bounding periph pulses |
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Patent Ductus Arteriosus (PDA)
Dx |
Small -
often no signs of cardiomeg Large - echocardio - LA & LV enlarged EKG - LVH CXR - cardiomegaly color-flow doppler- diagnostic |
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Patent Ductus Arteriosus (PDA)
Tx |
Indomethacin
(unless need PDA for survival) if indomethacin fails or child > 6-8 mos. old - surgical closure preferred |
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Coarctation of the Aorta -
What is it |
Narrowing prox or distal to DA
=> inc. flow above, dec. flow below coarctation more common in males Turner's 25% have bicuspid aortic valve |
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Coarctation of the Aorta -
Hx/PE |
Hx -
often presents in childhood with asymp HTN headache syncope epistaxis DOE claudication PE - systolic BP higher in upr ext may be gtr in right arm femoral pulses weak or delayed late systolic murmur in left axilla apical impulse forceful advanced cases - well-developed upper body lwr ext wasting |
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Coarctation of the Aorta -
Dx |
EKG - LVH
echocardiography color-flow doppler CXR - "3" sign rib notching aortography - diagnostic (cardiac catheterization) |
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Coarctation of the Aorta -
Tx |
Surgery or
balloon angioplasty endocarditis prophylaxis |
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Transposition of the
Great Arteries - What is it |
Pulmon & sys circ in parallel
aorta connected to RV pulmon artery connected to LV incompatible with life unless septal defect or PDA risk factors - babies of DM moms Apert's syn Down's cri-du-chat Trisomies 13 & 18 |
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Transposition of the
Great Arteries - Hx/PE |
Critically ill
cyanosis immed after birth tachypnea prog. resp failure CHF (some pts.) |
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Transposition of the
Great Arteries - Dx |
Echocardiography
CXR - "egg-shaped sihouette" "egg on a string" "apple on a string" |
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Transposition of the
Great Arteries - Tx |
Prostaglandin E1 (PGE1) -
to keep PDA open balloon atrial septostomy arterial or atrial switch op |
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Tetralogy of Fallot -
What is it |
VSD
pulmonary stenosis RVH overriding aorta rt-to-lt shunting => early cyanosis risk factors - Down's cri-du-chat Trisomies 13 & 18 |
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Tetralogy of Fallot -
Hx/PE |
Hx -
cyanosis dyspnea fatigability profound cyanosis = tet spell squatting for relief hypoxemia => FTT, mental status changes PE - SEM at left sternal border RV lift single S2 CHF signs possible |
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Tetralogy of Fallot -
Dx |
Echocardiography
catheterization CXR - boot-shaped heart dec. pulmon vascular markings EKG - rt-axis deviation RVH |
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Tetralogy of Fallot -
Tx |
PGE1 - keep reopen PDA
for cyanotic spells - O2 propanolol knee-chest position fluids morphine balloon atrial septostomy before surgical correction |
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Cerebral Palsy (CP) –
What is it |
Group of nonprogressive, nonhereditary neurological d/o
d/o in movement and posture MC movement d/o in kids MCC unknown - prenatal, perinatal and post insults risk factors – prematurity perinatal asphyxia intraut. growth retardation early infection or trauma brain malformation neonatal cerebral hemorrh |
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Cerebral Palsy (CP) –
What are the categories |
Spastic (pyramidal) -
spastic paresis of any limb 75% of cases MR up to 90% athetoid - extrapyramidal, b. ganglia uncontrollable jerking writhing worse with stress disappears during sleep ataxic - cerebellum hard to coordinate movement wide-based gait mixed |
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Cerebral Palsy (CP) –
Hx/PE |
May be associated with -
seizure d/o behavioral d/o hearing or vision impaired learning disabilities speech deficits hyperreflexia Babinski inc. tone/contractures weakness underdevelopment toe walking scissor gait hip dislocations scoliosis |
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Cerebral Palsy (CP) –
Dx |
Clinical
r/o metabolic d/o, cerebellar dysgenesis, spinocerebellar degen EKG (if seizures) |
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Cerebral Palsy (CP) –
Tx |
Special ed
physical therapy braces surgical rel of contractures for spasticity - diazepam dantrolene baclofen for severe contractures - baclofen pumps posterior rhizotomy |
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Febrile Seizures -
What is it |
In kids 6 mos. - 6 y/o
no evidence of intracranial infection or other cause risk factors - rapid rise in temp Hx in close relative |
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Febrile Seizures -
Hx/PE |
Most are simple seizures
simple - high fever fever onset within hrs of sz generalized seizure lasts < 15 min. 1 in a 24-hr period complex - low-grade fever fever for several days before seizure onset seizure has focal features can have postictal paresis lasts > 15 min. > 1 in a 24-hr period |
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Febrile Seizures -
Dx |
Find source of infection
LP - if signs of CNS infection No labs if presentation consistent with febrile sz Atypical presentation - electrolytes glucose BC UA CBC with diff EEG & MRI - complex seizures |
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Febrile Seizures -
Tx |
Simple -
aggressive antipyretics tx underlying illness complex - thorough neuro exam chronic anticonvulsants may be necessary |
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Febrile Seizures -
Complications |
Febrile Sz will recur in 30%
no inc. risk of epilepsy, developmental, intellectual or growth abnorm those with complex seizures - 10% risk of dev. epilepsy |
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Neonatal Jaundice -
What is it What are the types What is kernicterus |
Inc. serum bilirubin
from inc. production or dec. excretion conjugated - always pathologic uncon - patholog or physiolog physiologic jaundice - not present until 72 hrs after birth bilirubin peaks < 15 mg/dL resolves by 1 wk in term resolves by 2 wks in premies pathologic jaundice - present in 1st 24 hrs of life bilirubin rises to > 15 mg/dL persists past 1 wk in term persists past 2 wks in premies kernicterus - unconjug hyperbilirubinemia bilirubin deposits in pons, basal ganglia, cerebellum irreversible can be fatal risk factors - premies asphyxia sepsis |
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Neonatal Jaundice -
Hx/PE |
Hx -
child breastfed or formula? intrauterine drugs fam Hx of - hemoglobinopathies enzyme def. RBC defects Sxs - abdom distention delayed passage of meconium light-colored stools dark urine low Apgar scores weight loss vomiting kernicterus - lethargy poor feeding high-pitched cry hypertonicity seizures jaundice may be cephalopedal check for signs of - infection congen malformations cephalohematomas bruising pallor petechiae hepatomegaly |
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Neonatal Jaundice -
Dx |
CBC
periph blood smear blood type mom and baby Coombs' test bilirubin levels direct hyperbilirubinemia - LFTs bile acids BC sweat test tests for aminoacidopathies & a1-antitrypsin deficiency Sepsis w/u and ICU - jaundice febrile hypotensive and/or tachypneic |
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Neonatal Jaundice -
Tx |
Tx underlying cause
unconjugated - severe - exchange transfusion if mild - phototherapy start earlier for premies (start at 10-15 mg/dL) |
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Down Syndrome -
What is it |
Trisomy 21
MC chromosome d/o #2 cause of congen MR risk inc. with mom's age, but 80% of kids are born to women < 35 y/o flat facial profile prominent epicanthal folds simian crease dec. levels of AFP brushfield spots duodenal atresia - double bubble on US/XR congen heart disease - septum primum-type ASD due to endocardial cushion defect Alzheimer's > 35 y/o inc. risk of ALL meiotic nondisjunction |
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Edwards' Syndrome -
What is it |
Trisomy 18 (election age=18)
severe MR rocker bottom feet low-set ears micrognathia congen heart dis clenched hands prominent occiput death usu < 1 y/o |
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Patau's Syndrome -
What is it |
Trisomy 13 (puberty=13)
severe MR microphthalmia microcephaly cleft lip/palate abnorm forebrain polydactyly congen heart disease death usu < 1 y/o |
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Klinefelter's Syndrome -
What is it |
XXY (male)
inactivated X (Barr body) 1 of MCC of male hypogonadism testicular atrophy eunuchoid body shape, long long extremities gynecomastia female hair distribution |
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Turner' Syndrome -
What is it |
XO (No Barr body)
short stature ovarian dysgenesis webbing of neck cystic hygroma coarctaton of aorta MCC of primary amenorrhea |
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Double Y males -
What is it |
XYY
phenotypically normal very tall severe acne antisocial behavior |
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Phenylketonuria (PKU) -
What is it |
Phenylalanine => tyrosine
In PKU, dec. phenylalanine hydroxylase or tetrahydrobiopterin cofactor tyrosine becomes essential phenylalanine builds up => excess phenylketones phenylketones - phenylacetate phenyllactate phenylpyruvate MR fair skin eczema musty body odor screened for at birth Tx - dec. phenylalanine (in Nutrasweet) and inc. tyrosine in diet |
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Fabry's Disease -
(lysosomal storage disease) What is it |
X-linked recessive
def. of a-galactosidase A ceramide trihexoside accums renal failure |
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Krabbe's Disease -
(lysosomal storage disease) What is it |
AR (autosomal recessive)
def. of B-galactosidase galactocerebroside accums in the brain optic atrophy spasticity early death |
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Gaucher's Disease -
(lysosomal storage disease) What is it |
AR
def. of B-glucocerebrosidase glucocerebroside accums in brain, liver, spleen, bm Gaucher's cells - "crinkled paper" enlarged cytoplasm Type I - more common, compa- tible with normal life span |
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Niemann-Pick Disease -
(lysosomal storage disease) What is it |
AR
def. of sphingomyelinase sphingomyelin and cholesterol build up in reticuloendo- thelial & parenchymal cells cherry-red spot on macula death by age 3 |
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Tay-Sachs Disease -
(lysosomal storage disease) What is it |
AR
absence of hexosaminidase A GM2 ganglioside accums cherry-red spot on macula death by age 3 MC lysosomal storage disease that causes MR |
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Metachromatic leukodystrophy -
(lysosomal storage disease) What is it |
AR
def. of arylsulfatase A sulfatide accums in brain, kidney, liver, periph n. |
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Hurler's Syndrome -
(lysosomal storage disease) What is it |
AR
def. of a-L-iduronidase corneal clouding MR |
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Hunter's Syndrome -
(lysosomal storage disease) What is it |
X-linked recessive
def. of iduronate sulfatase mild form of Hurler's no corneal clouding mild MR |
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Fragile X Syndrome -
What is it |
X-linked
3rd MCC of MR FMR1 gene affected anticipation triple repeat of CGG autism large testes, jaw, ears floppy/prolapsed mitral valve |
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APGAR Score -
Chart |
Appearance -
skin color 0 = blue all over 1 = blue at extremities 2 = normal Pulse - 0 = none 1 = < 100 2 = > 100 Grimace - reflex irritability 0 = none 1 = grimace, feeble cry 2 = sneeze, cough, pull away Activity - muscle tone 0 = none 1 = some flexion 2 = active movement Respiration - 0 = none 1 = weak or irreg 2 = strong |
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APGAR Score -
What do total scores mean |
Score at 1 min. after birth
then at 5 min. score 8-10 - good cardiopulm adaptation score 4-7 - possible need for resus observe stimulate possible need for vent support score 0-3 - resus immed |
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Erythema Infectiosum -
(Fifth Disease) Cause Characteristics |
Cause -
parvovirus B19 prodrome none fever absent or low-grade "slapped cheek" erythematous pruritic maculopapular rash goes to arms spreads to trunk and legs worse with fever and sun |
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Erythema Infectiosum -
(Fifth Disease) Complications |
Arthritis
hemolytic anemia - aplastic crisis in sickle cell encephalopathy associated c hydrops fetalis |
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Measles -
Cause Characteristics |
Paramyxovirus
prodrome - low-grade fever conjunctivitis coryza cough Koplik's spots - buccal mucosa after 1-2 days maculopap rash from ears down |
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Measles -
Complications |
Giant cell pneumonia
otitis media laryngotracheitis rare - subac scleros. panencephalitis |
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Rubella -
Cause Characteristics |
Rubella virus
prodrome - asymp or tender, generalized lymphadenopathy erythematous, tender, maculopapular rash slight fever polyarthritis in adolescents |
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Rubella -
Complications |
Encephalitis
thrombocytopenia congen infections associated with congen anomalies |
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Roseola Infantum -
Cause Characteristics |
HHV-6
prodrome - acute onset of high fever no other Sxs for 3-4 days maculopap rash as fever breaks starts on trunk => face and extremities often lasts < 24 hrs |
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Roseola Infantum -
Complications |
Rapid fever onset =>
febrile seizures |
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Rotavirus -
Characteristics |
Primary cause of diarrhea
in kids < 2 fever and vomiting then diarrhea upper resp Sxs lasts < 1 wk infection confirmed by Elisa oral rehydration sufficient |
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Varicella -
Cause Characteristics |
VZV
prodrome - mild fever anorexia malaise precedes rash by 24 hrs generalized, pruritic, "teardrop" vesicular rash starts on trunk spreads to periphery lesions often at different stages of healing infectious from 24 hrs before eruption til lesions crust over |
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Varicella -
Complications |
In immunocompromised kids -
progressive varicella with meningoencephalitis and hepatitis congen infections => congen anomalies |
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Varicella Zoster -
Cause Characteristics |
Prodrome -
reactivation of Varicella infection starts as pain along affected sensory n. pruritic "teardrop" vesicular rash in dermatomal distribution uncommon unless immunocomp |
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Varicella Zoster -
Complications |
Encephalopathy
aseptic meningitis pneumonitis TTP Guillain-Barre cellulitis arthritis |
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Hand-Foot-and-Mouth Disease -
Cause Characteristics |
Coxsackie A
prodrome - fever anorexia oral pain rash - oral ulcers maculopap vesicular rash on hands, feet, buttocks |
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Hand-Foot-and-Mouth Disease -
Complications |
None
(self-limited) |
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Tracheoesophageal Fistula -
What is it |
Tract between trachea & esoph
associated with esoph atresia & VACTERL anomalies - vertebral anal cardiac tracheal esophagus renal limb |
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Tracheoesophageal Fistula -
Caused by Presentation |
Polyhydramnios in utero
inc. oral secretion inability to feed gagging resp distress |
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Tracheoesophageal Fistula -
Dx |
CXR after NGT
air in GI tract bronchoscopy - to confirm |
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Tracheoesophageal Fistula -
Tx |
Surgical repair
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Congenital Diaph Hernia -
What is it |
GI tract segment protrudes
thru diaph into thorax 90% are post. lt. Bochdalek |
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|
Congenital Diaph Hernia -
Presentation |
Resp distress from -
pulmonary hypoplasia pulmonary HTN sunken abdomen bowel sounds over lt. hemithorax |
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Congenital Diaph Hernia -
Dx |
US in utero
postnatal CXR - to confirm |
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Congenital Diaph Hernia -
Tx |
Hi-freq ventilation or
extracorporeal membrane oxygenation (ECMO) (to manage pulmonary HTN) surgical repair |
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Gastroschisis -
What is it |
Herniation of intestine
thru abdom wall next to umbilicus (usually on right) with no sac |
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|
Gastroschisis -
Caused by Associated with |
Polyhydramnios in utero
often premie associated with - GI stenoses GI atresia |
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Gastroschisis -
Tx |
Surgical emergency
single-stage closure possible in only 10% |
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Omphalocele -
What is it |
Herniation of abdom viscera
thru abdom wall at umbilicus into sac covered by peritoneum and amniotic memb |
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|
Omphalocele -
Caused by Associated with |
Polyhydramnios in utero
often premie associated with other GI & cardiac defects |
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|
Omphalocele -
Tx |
C-section -
to prevent sac rupture if sac intact - postpone surg correction until pt. fully resuscitated keep sac covered/stable with petroleum & gauze intermittent NG suction - to prevent abdom distention |
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Duodenal Atresia -
What is it |
Complete or partial failure
of duodenal lumen to recanalize during gestational wks 8-10 |
|
|
Duodenal Atresia -
Presentation Caused by Associated with |
Bilious emesis within hrs
after 1st feeding polyhydramnios in utero associated with - Down's other cardiac/GI anomalies: annular pancreas malrotation imperforate anus |
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|
Duodenal Atresia -
Dx |
Double-bubble sign on XR
(prox to site of atresia) |
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Duodenal Atresia -
Tx |
Surgical repair
|
|
|
Meckel's Diverticulum -
What is it |
MC congen GI tract anomaly
vestigial remnant of omphalomesenteric duct rule of 2's - 2x's as many males 2 ft. from ileocecal valve 2% of people affected 2 types of mucosa - gastric pancreatic |
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Meckel's Diverticulum -
Presentation |
MC presentation -
painless rectal bleeding painful diverticulitis intest. obstruction from - intussusception or volvulus |
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Meckel's Diverticulum -
Dx |
Meckel's scan -
for ectopic gastric mucosa uses IV technetium pertechnetate |
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Meckel's Diverticulum -
Tx |
Surgery
|
|
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Hirschsprung's Disease -
(Congen Aganglionic Megacolon) What is it |
Absence of autonomic
innervation of bowel wall inadeq relaxation and peristalsis => intest. obstruction |
|
|
Hirschsprung's Disease -
(Congen Aganglionic Megacolon) Presentation |
Abdom distention
bilious vomiting fail to pass meconium in 1st 24 hrs of life |
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Hirschsprung's Disease -
(Congen Aganglionic Megacolon) Dx |
Barium enema -
dilated prox segment narrowed distal segment rectal Bx - to confirm |
|
|
Hirschsprung's Disease -
(Congen Aganglionic Megacolon) Tx |
Colostomy prior to
corrective surgery |
|
|
Hypospadias -
What is it |
Abnorm urethral opening
on ventral surface of penis due to incomplete dev of distal urethra |
|
|
Hypospadias -
Presentation Associated with |
Chordee
associated with - hernias cryptorchidism |
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|
Hypospadias -
Tx |
Circumcision contraindicated
surgical repair uses preputial tissue |
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X-linked Agammaglobulinemia
(Bruton's Disease) - What is it |
B-cell def.
boys only may present < 6 mos. of age at risk for life-threatening Pseudomonas infections |
|
|
X-linked Agammaglobulinemia
(Bruton's Disease) - Dx |
No B cells
low levels of all Ab classes |
|
|
X-linked Agammaglobulinemia
(Bruton's Disease) - Tx |
IVIG
prophylactic ABx |
|
|
Common Variable Immunodeficiency -
What is it |
Ig levels drop in
2nd-3rd decade of life inc. risk of lymphoma inc. risk of autoimmune dis. |
|
|
Common Variable Immunodeficiency
Dx |
Ig levels
antibody titers |
|
|
Common Variable Immunodeficiency
Tx |
IVIG
prophylactic ABx |
|
|
IgA Deficiency -
What is it |
MC immunodeficiency
usually asymptomatic may have recurrent infections |
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|
DiGeorge Syndrome
(Thymic aplasia) - What is it |
CATCH-22
Cardiac defects Abnormal facies Thymic hypoplasia Cleft palate Hypocalcemia 22 - microdeletions in chrom22 tetany in first days of life |
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|
DiGeorge Syndrome
(Thymic aplasia) - Dx |
Absolute lymphocyte count,
mitogen stimulation response and delayed hypersensitivity skin testing |
|
|
DiGeorge Syndrome
(Thymic aplasia) - Tx |
BMT - if severe
IVIG thymus transplant |
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|
Ataxia-Telangiectasia -
What is it |
DNA repair defect
oculocutaneous telangiectasias progressive cerebellar ataxia |
|
|
Ataxia-Telangiectasia
Tx |
No effective Tx for CNS abnorm
neuro deterioration progresses death by 30 y/o |
|
|
Severe Combined
Immunodeficiency (SCID) - What is it |
Severe lack of B & T cells
freq. severe bact. infections chronic Candidiasis opportunistic organisms |
|
|
Severe Combined
Immunodeficiency (SCID) - Tx |
BMT or stem cell transplant
IVIG PCP prophlaxis until BMT gene therapy may be future option |
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|
Wiskott-Aldrich Syndrome -
What is it |
X-linked recessive
T & B cell dysfunction thrombocytopenia small-sized platelets eczema high IgE high IgA low IgM bloody diarrhea bleeding gums prolonged nosebleeds |
|
|
Wiskott-Aldrich Syndrome -
Tx |
Protective helmet
IVIG aggressive ABx for infections HLA-identical BMT if no BMT - rarely survive to adulthood |
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|
Chronic Granulomatous Disease-
(CGD) What is it |
X-linked or AR
deficient superoxide production by PMNs & M0s usual sites of infection - skin lungs (pneumonia) lymph nodes liver (abscesses, hepatitis) bones (osteomyelitis) swollen collections of infected tissue obstruct intestines (IBD) and urinary tract (UTIs) |
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|
Chronic Granulomatous Disease-
(CGD) Dx |
Absolute neutrophil count
and adhesion assays - chemotaxic phagocytic bactericidal diagnostic - neg. nitroblue tetrazolium dye reduction test (NBT) |
|
|
Chronic Granulomatous Disease-
(CGD) Tx |
Daily TMP-SMX
judicious ABx use during infections IFN-g - can dec. incidence of serious infection |
|
|
Chediak-Higashi -
What is it |
AR
giant lysosomal granules dev. in neutrophils lysosomes can't fuse with phagosomes => ingested bact. can't be lysed oculocutaneous albinism neuropathy neutropenia |
|
|
C1 Esterase (Inhib) Def. -
(Hereditary Angioedema) What is it |
C1 inhibitor -
acute phase protein inhibits proteinases of: complement pathway clotting pathway kinin generator pathway fibrinolytic pathway deficiency => her. angioedema AD can affect - hands & feet - local edema bowel - extreme abdom pain mouth airway - life-threat. edema usually lasts 3 days can be precip by trauma, virus aggravated by stress |
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|
C1 Esterase (Inhib) Def. -
(Hereditary Angioedema) Dx |
Total hemolytic C' assay- CH50
if defect in one component - no CH50 reduction then det. which component |
|
|
C1 Esterase (Inhib) Def. -
(Hereditary Angioedema) Tx |
Daily prophylactic danazol
purified C1 esterase and FFP - prior to surgery |
|
|
Terminal Complement Deficiency
(C5-C9) - What is it |
AR
recurrent N. meningitidis & dissem. gonorrhea infections rarely - systemic lupus |
|
|
Terminal Complement Deficiency
(C5-C9) - Tx |
Meningococcal vaccine
appropriate ABx |
|
|
Hyper IgM Syndrome -
What is it |
MC - def. in CD40 ligand
in T Helper => can't class switch normal or high IgM low IgG, IgA, IgE XL recessive (most common) AR (others) severe upr & lwr respiratory diarrhea - Cryptosporidium |
|
|
Hyper IgM Syndrome -
Tx |
IVIG
PCP prophylaxis - TMP-SMX |
