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Pediatrics Flashcards

1
Q

presentation of systemic onset juvenile idiopathic arthritis

A

> 2 weeks of fever once a day, 1+ jt arthritis, pink macular rash that worsens during fever

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2
Q

tricuspid valve atresia findings

A

cyanotic infant with L axis deviation. absent R waves in precordial leads, peaked P waves, decreased pulmonary marking on CXR

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3
Q

scarlet fever presentation

A

fever, pharyngitis, sandpaper like rash, circumoral pallor, and strawberry tongue

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4
Q

Wiskott Aldrich syndrome

A

eczema + thrombocytopenia + hypogammglobulinemia

X linked

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5
Q

Fanconis anemia

A

AR, average dx age = 8
progressive pancytopenia + macrocytosis
ass’d w/ cafe au last spots, microcephaly, microphthalmia, short stature, horseshoe kidney, absent thumbs

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6
Q

Diamond Blackfan syndrome

A

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
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7
Q

trachoma

A

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)
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8
Q

management of newborns born to mother with active Hep B

A

Hep B Ig followed by Hep B vaccine

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9
Q

which kids with bronchiolitis get palivizumab?

A

preterm birth <29 weeks gestation
chronic lung disease of prematurity
hemodynamically significant CHD

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10
Q

retropharyngeal abscess

A

feevr + dysphagia + inability to extend neck + muffled voice + widened prevertebral space on lateral Xray

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11
Q

Friedreich ataxia

A

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

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12
Q

risk factors for jejunal atresia?

A

prenatal exposure to cocaine and other vasoconstrictive drugs
meconium ileus that causes volvulus and necrosis of bowel

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13
Q

erythema chronic migrans

A

early, localized Lyme disease rash
tx = oral doxy, amor, or cefuroxime
**amox if <8 yo

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14
Q

Guillain barre syndrome management

A
  • *serial measurements of FVC to monitor respiratory function to predict impeding resp failure and intubation need
  • *cardiopulm support
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15
Q

classic presentation of galactosemia

A

–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

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16
Q

galactokinase deficiency presentation

A

asym except cataracts

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17
Q

complications of SGA infants (6)

A
  1. hypoxia, perinatal asphyxia,
  2. meconium aspiration
  3. hypothermia
  4. hypoglycemia
  5. hypocalcemia
  6. polycythemia (from increased EPO secretion in response to hypoxia)
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18
Q

RDS risk factors

A
  1. premie
  2. male sex
  3. perinatal asphyxia
  4. maternal DM
  5. C section w/o labor
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19
Q

McCune Albright syndrome

A

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

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20
Q

bug for PNA in CF patients

A

childhood: staph aureus
adults (20+): pseudomonas

Always treat for both

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21
Q

edema in Turner’s syndrome

A

due to dysgenesis of lymphatic system –> congenital lymphedema
**nonpitting

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22
Q

what fluids should be used for IV fluid resuscitation?

A

NS (isotonic)

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23
Q

what is the most common predisposing factor for orbital cellulitis?
complications?

A

bacterial sinusitis

complications: blind, abscess, cavernous sinus thrombosis, intracranial infection, death

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24
Q

most common CHD in Down syndrome?

A

complete atrioventricular septal defect

-load S2
-systolic ejection murmur (b/c incr pulm flow from ASD)
+/- holosystolic VSD murmur

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25
Q

spondylolisthesis

A

forward slip of vertebrae (usually L5 over S1) usually in preadolescent kids
–back pain + neuro dysfunction + palpable step off

26
Q

von Gierke disease (type 1 glycogen storage disease)

A

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
27
Q

what to do if adverse run to tDap?

A

give DT instead

-reaction is to pertussis component

28
Q

blount disease

A

genu varum/bow legs in >2yo

ortho consult

29
Q

legg calve perthes disease

A

avascular necrosis of femoral head
painless limp in boy <10yo
ortho consult

30
Q

slipped capital femoral epiphysis

A

painful limp in obese boy

ortho consult

31
Q

osgood schlatter disease

A

overuse injury of quads and patellar tendon –> traction apophysitis of tibial tuberosity –> localized pain and swelling
tx = RICE and NSAIDs

32
Q

lichen planus

A

purple polygonal pruritic papules

tx = PO steroids

33
Q

Henoch Schonlein purpura

A

anaphylactoid
IgA mediated small vessel vasculitis –> palpable purport on butt and legs + renal disease + ileocecal edema –> colicky abdominal pain and risk for intussusception

34
Q

porphyria cutanea tardis

A

mut uroporphobilinogen decarboxylase –> photosensitivity to UV light + tea colored urine + recurrent attacks of abdominal pain

35
Q

alopecia areata

A

hair stops growing suddenly –> small patches of complete hair loss with exclamation point stubs

36
Q

cherry red macula w/o HSM

A

Niemann pick disease

mut spingomyelinase

37
Q

retained primary teeth

A

job syndrome (hyper IgE)

38
Q

single central incisor

A

GH deficiency

39
Q

pes cavus

A

high arched foot ass’d with freidrich ataxia and Charcot Marie tooth disease

40
Q

micropenis

A

GH deficiency

41
Q

neonatal dx of HIV

A

PCR b/c mom’s Ab can cause false +

42
Q

PPx for cat/dog bite

A

Augmentin

43
Q

salmon patch (nevus flammeus)

A

benign splotchy red rash on forehead, eyelids, back of neck of newborn
–more prominent with exercise or emotion

44
Q

pustular melanosis

A

benign little pustules that leave a hyper pigmented spot when ruptured

45
Q

erythema toxicum

A

benign yellow-white papule with surrounding erythema

–fluid exam shows eos

46
Q

sebaceous nevus

A

raised yellow orange hairless lesion on scalp

tx = resection before adolescent b/c it can undergo malignant transformation

47
Q

incontinentia pigmenti

A

XR
inflam bullae that evolve into hyper pigmented lesions
lethal in males

48
Q

transient tachypnea of the newborn

A

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

49
Q

congenital varicella syndrome

A

limb hypoplasia + clear vesicles –> cutaneous scars, chorioretinitis, cortical atrophy
Tx VZIG if mom has chickenpox 5 days prior - 2 days after delivery; otherwise reassurance

50
Q

caput succedaneum

A

soft tissue swelling of scalp where baby was delivered

crosses midline

51
Q

cephalohematoma

A

bleedings between periosteum and skull –> squishy feel to scalp that doesn’t cross midline

52
Q

subgaleal hemorrhage

A

bleeding under gall aponeurosis –> squishy feel to rapidly expanding scalp that crosses midline
tx = admit to NICU for possible hemorrhagic shock

53
Q

vit A toxicity

A

inc CSF pressure (HA, blurry vision)
dr skin
teratogenic
found in animal liver consumption

54
Q

vitamin B7 / biotin deficiency

A

seborrheic dermatitis
enteritis
alopecia
*found in kids who eat raw eggs or biotinidase deficiency

55
Q

vit C toxicity

A

uric acid stones

56
Q

vit E deficiency nd toxicity

A 
deficiency = hemolytic anemia
toxicity = hemorrhagic diathesis
57
Q

what important nutrient is goats milk missing? cows?

A 
goat = folate
cow = iron
58
Q

management of aspirin poisoning

A

sodium bicarb +/- dialysis

59
Q

cyanide poisoning and tx

A

headache –> confusion, agitation –> seizures and meta alka

tx = nitrites, hydroxocobalamin + thiosulfate

60
Q

phenothiazine and metoclopramide poisoning

A

EPS (torticollis, opisthotonus, dyspahgia)

tx = diphenhydramine

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