Pediatrics Flashcards
presentation of systemic onset juvenile idiopathic arthritis
> 2 weeks of fever once a day, 1+ jt arthritis, pink macular rash that worsens during fever
tricuspid valve atresia findings
cyanotic infant with L axis deviation. absent R waves in precordial leads, peaked P waves, decreased pulmonary marking on CXR
scarlet fever presentation
fever, pharyngitis, sandpaper like rash, circumoral pallor, and strawberry tongue
Wiskott Aldrich syndrome
eczema + thrombocytopenia + hypogammglobulinemia
X linked
Fanconis anemia
AR, average dx age = 8
progressive pancytopenia + macrocytosis
ass’d w/ cafe au last spots, microcephaly, microphthalmia, short stature, horseshoe kidney, absent thumbs
Diamond Blackfan syndrome
macrocytic pure red cell aplasia ass’d with congenital abnormalities (short stature, webbed neck, cleft lip, shield chest, triphalangeal thumbs)
- -low retic (b/c intrinsic defect of RBC progenitors)
- -no PMN nucleus hyperseg
- -elev fetal Hb on electrophoresis
- -Tx = steroids +/- transfusion
trachoma
Due to Chlamydia trachomatis ABC
- -follicular conjunctivitis + pannus/neovasc in cornea –> eyelid scarring and inversion of eyelashes –> ulceration –> blindness
- -often presents with concurrent nasopharyngeal infection
- -treat the entire village (azith)
management of newborns born to mother with active Hep B
Hep B Ig followed by Hep B vaccine
which kids with bronchiolitis get palivizumab?
preterm birth <29 weeks gestation
chronic lung disease of prematurity
hemodynamically significant CHD
retropharyngeal abscess
feevr + dysphagia + inability to extend neck + muffled voice + widened prevertebral space on lateral Xray
Friedreich ataxia
AR, excess GAA repeats –> abnorm frataxin protein
neuro (ataxia, dysarthria, loss of position sense and reflexes)
+ skeleteal (scoliosis, hammer toes/feet deformities)
+ cardiac (concentric hypertrophic cardiopmyopathy, degeneration of cardiac muscle fibers -> can cause T wave inversion)
MRI - atrophy of (cervical?) SC and minimal cerebellar atrophy
death-heart and resp complications
TX = PT, psych support
risk factors for jejunal atresia?
prenatal exposure to cocaine and other vasoconstrictive drugs
meconium ileus that causes volvulus and necrosis of bowel
erythema chronic migrans
early, localized Lyme disease rash
tx = oral doxy, amor, or cefuroxime
**amox if <8 yo
Guillain barre syndrome management
- *serial measurements of FVC to monitor respiratory function to predict impeding resp failure and intubation need
- *cardiopulm support
classic presentation of galactosemia
–galactose 1 P uridyl transferase deficiency –> elevated blood levels of galactose
infant/newborn with failure to thrive, bilateral cataracts, jaundice, hypoglycemia
+/-aminoaciduria, hepatic cirrhosis, mental retardation
*increased risk for E coli sepsis
galactokinase deficiency presentation
asym except cataracts
complications of SGA infants (6)
- hypoxia, perinatal asphyxia,
- meconium aspiration
- hypothermia
- hypoglycemia
- hypocalcemia
- polycythemia (from increased EPO secretion in response to hypoxia)
RDS risk factors
- premie
- male sex
- perinatal asphyxia
- maternal DM
- C section w/o labor
McCune Albright syndrome
3 P’s:
Precocious puberty
+ Pigmentation (cafe au lait syndrome)
+ Polyostotic fibrous dysplasia (bone defects)
+/- endocrine disorders (hyperthyroidism, prolactin or GH secreting pituitary adenomas, adrenal hypercortisolism)
*sporadic defect
bug for PNA in CF patients
childhood: staph aureus
adults (20+): pseudomonas
Always treat for both
edema in Turner’s syndrome
due to dysgenesis of lymphatic system –> congenital lymphedema
**nonpitting
what fluids should be used for IV fluid resuscitation?
NS (isotonic)
what is the most common predisposing factor for orbital cellulitis?
complications?
bacterial sinusitis
complications: blind, abscess, cavernous sinus thrombosis, intracranial infection, death
most common CHD in Down syndrome?
complete atrioventricular septal defect
-load S2
-systolic ejection murmur (b/c incr pulm flow from ASD)
+/- holosystolic VSD murmur
spondylolisthesis
forward slip of vertebrae (usually L5 over S1) usually in preadolescent kids
–back pain + neuro dysfunction + palpable step off
von Gierke disease (type 1 glycogen storage disease)
glucose 6 phosphatase deficiency impairs glycogen to glucose conversion –> glycogen accumulates in affected organs
- -presents at 3-4 months
- -hypoglycemia (+/- seizures) + lactic acidosis + hyperuricemia + hyperlipidemia
- -doll like face, thin extremities, short stature, protuberant abdomen (b/c hepatomegaly)
- -spleen and heart not involved
what to do if adverse run to tDap?
give DT instead
-reaction is to pertussis component
blount disease
genu varum/bow legs in >2yo
ortho consult
legg calve perthes disease
avascular necrosis of femoral head
painless limp in boy <10yo
ortho consult
slipped capital femoral epiphysis
painful limp in obese boy
ortho consult
osgood schlatter disease
overuse injury of quads and patellar tendon –> traction apophysitis of tibial tuberosity –> localized pain and swelling
tx = RICE and NSAIDs
lichen planus
purple polygonal pruritic papules
tx = PO steroids
Henoch Schonlein purpura
anaphylactoid
IgA mediated small vessel vasculitis –> palpable purport on butt and legs + renal disease + ileocecal edema –> colicky abdominal pain and risk for intussusception
porphyria cutanea tardis
mut uroporphobilinogen decarboxylase –> photosensitivity to UV light + tea colored urine + recurrent attacks of abdominal pain
alopecia areata
hair stops growing suddenly –> small patches of complete hair loss with exclamation point stubs
cherry red macula w/o HSM
Niemann pick disease
mut spingomyelinase
retained primary teeth
job syndrome (hyper IgE)
single central incisor
GH deficiency
pes cavus
high arched foot ass’d with freidrich ataxia and Charcot Marie tooth disease
micropenis
GH deficiency
neonatal dx of HIV
PCR b/c mom’s Ab can cause false +
PPx for cat/dog bite
Augmentin
salmon patch (nevus flammeus)
benign splotchy red rash on forehead, eyelids, back of neck of newborn
–more prominent with exercise or emotion
pustular melanosis
benign little pustules that leave a hyper pigmented spot when ruptured
erythema toxicum
benign yellow-white papule with surrounding erythema
–fluid exam shows eos
sebaceous nevus
raised yellow orange hairless lesion on scalp
tx = resection before adolescent b/c it can undergo malignant transformation
incontinentia pigmenti
XR
inflam bullae that evolve into hyper pigmented lesions
lethal in males
transient tachypnea of the newborn
tachypnea, grunting, +/- cyanosis in c/s baby due to retained fetal lung fluid
CXR - palm vascular markings
Tx - 100% oxy (improves sats unlike RDS)
Resolves within 3 days
congenital varicella syndrome
limb hypoplasia + clear vesicles –> cutaneous scars, chorioretinitis, cortical atrophy
Tx VZIG if mom has chickenpox 5 days prior - 2 days after delivery; otherwise reassurance
caput succedaneum
soft tissue swelling of scalp where baby was delivered
crosses midline
cephalohematoma
bleedings between periosteum and skull –> squishy feel to scalp that doesn’t cross midline
subgaleal hemorrhage
bleeding under gall aponeurosis –> squishy feel to rapidly expanding scalp that crosses midline
tx = admit to NICU for possible hemorrhagic shock
vit A toxicity
inc CSF pressure (HA, blurry vision)
dr skin
teratogenic
found in animal liver consumption
vitamin B7 / biotin deficiency
seborrheic dermatitis
enteritis
alopecia
*found in kids who eat raw eggs or biotinidase deficiency
vit C toxicity
uric acid stones
vit E deficiency nd toxicity
deficiency = hemolytic anemia toxicity = hemorrhagic diathesis
what important nutrient is goats milk missing? cows?
goat = folate cow = iron
management of aspirin poisoning
sodium bicarb +/- dialysis
cyanide poisoning and tx
headache –> confusion, agitation –> seizures and meta alka
tx = nitrites, hydroxocobalamin + thiosulfate
phenothiazine and metoclopramide poisoning
EPS (torticollis, opisthotonus, dyspahgia)
tx = diphenhydramine
