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*****aPeds > Misc peds > Flashcards

Misc peds Flashcards

1
Q

At about what age does the infant lose the mother’s protective antibodies?

A

~ 6 months

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2
Q

characteristics of CVID?

A

can have similar clinical picture to Bruton’s agammaglobulinemia, but has NORMAL B cell levels and presents in 20s or 30s

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3
Q

what would the serum/urine sodium osmolality be in a kid with diarrhea and vomiting?

A

low serum sodium
low serum osmolality
low urine Na
high urine Osm

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4
Q

MC cyanotic lesion presenting in immediate NB period?

A

transposition of the great arteries

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5
Q

What would you hear on PE with TGA?

A

normal PMI or parasternal heave

  • S2 loud and single
  • no murmurs or soft systolic ejection murmur at mid LSB
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6
Q

synonym for atopic dermatitis?

A

eczema

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7
Q

autoimmune polyglandular disease TYPE 1

A
  • hypoparathyroidism
  • Addison disease
  • mucocutaneous candidiasis
  • small number w/ autoimmune thyroiditis
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8
Q

autoimmune polyglandular disease TYPE 2

A

(Schmidt syndrome)

  • Addison disease
  • insulin-dependent DM
  • +/- thyroiditis
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9
Q

What are some HLA-DR3 related disorders?

A

Graves, addison, DM, MG, celiac

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10
Q

What HLA markers is graves assoc with?

A

HLA-B8 and DR3

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11
Q

What exactly is choanal atresia?

A

obliteration or blockage of posterior nasal aperture

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12
Q

best initial test for choanal atresia?

A

insert a catheter in nose

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13
Q

confirmatory test for choanal atresia?

A

CT scan w/ contrast of head

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14
Q

CHARGE

A
  • Coloboma
  • Heart defects
  • Atresia of the choanae
  • retardation (growth +/_ mental)
  • GU abn
  • ear anomalies
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15
Q

what is adrenoleukodystrophy?

A

X-linked; mutation prevents transport of VLCFA into peroxisomes, preventing their B-ox/breakdown

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16
Q

how does adrenoleukodystrophy present?

A
  • accum of abnormal VLCFA in organs (neurons and adrenal cortex)
  • neuro: weak/spastic -> dementia, blindness, quadriparesis
  • primary adrenal insufficiency: hypo Na, postural HoTN, hyper K, lack of response to cosyntropin test
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17
Q

full term infant w/ resp distress and decreased breath sounds on left. suspected dx?

A

congenital diaphragmatic hernia

  • scaphoid abdomen
  • intrathoracic air-filled intestine
  • PMI displaced right
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18
Q

1st step to tx congenital diaphragmatic hernia?

A

NG suction for bowel decompression, and immediate intubation

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19
Q

what is the range for impaired glucose tolerance?

A

> 100 and <126 FBG

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20
Q

when should inhaled steroids appropriate to tx asthma?

A

DOC for persistent asthma:

  • use B2 agonist 3x/week
  • symptomatic 3x/week
  • 2 exacerbations in 6 months requiring oral steroids
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21
Q

CXR features with TTN?

A
  • perihilar streaking (engorged lymphatic system w/ retained lung fluid)
  • fluid in fissures
  • > improves rapidly with supplemental O2, resolves in 2-3 days
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22
Q

findings with air leak syndrome?

A

(pneumomediastinum, PTX, pneumopericardium)

  • sudden onset resp distress
  • poss HoTN (dec CO), muffled heart tones, abd distention, asymm chest, deviated heart sounds
  • CXR: free air in hemithorax, visible edge of the collapsed lung
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23
Q

findings with meconium amniotic fluid aspiration?

A
  • resp distress w/in first hours
    CXR: patchy infiltrates, coarsely streaked bilateral lung fields, increased AP diameter, flattened diaphragm
  • don’t recover as rapidly as TTN
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24
Q

what complication of GAS is not preventable with early abx?

A

glomerulonephritis

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25
Q

how should subsequent pregnancies be managed if first born has CAH salt losing type?

A
  • give dexamethasone to mom beginning at 6 weeks gestation (crosses placenta, suppresses fetal adrenal steroid secretion, prevents masculinization)
  • CVS at 10-12 weeks to determine genotype ; continue tx if female
  • continue tx until genetic analysis confirms absence of abnormal genes
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26
Q

risk factors for developmental dysplasia of the hip (difficulty w/ abduction on barlow test)?

A
  • MC in females than males
  • breech delivery
  • first born child
  • family history
  • MC in whites/navajo than blacks
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27
Q

osteochondritis?

A

osgood schlatter

- stress rxn of patellar tendon insertion into tibial tubercle

28
Q

MCC of diffuse bronchiectasis in children?

A
  • CF

- often accompanied by chronic diarrhea and FTT

29
Q

pathophys of bronchiectasis?

A

recurrent infxn/inflamm -> chronic obs and hypertrophy of bronchi -> abn dilatation of bronchi and bronchioles

30
Q

growth deficiency, microcephaly, short palp fissures, maxillary hypoplasia, micrognathia, thin upper lip

A

fetal alcohol syndrome

31
Q

lithium exposure in pregnancy?

A

increased risk for CHD (Ebstein anomaly) but not others

32
Q

CNS defects (hydroceph, microceph), facial asymm, microtia/anotia, conotruncal cardiac abnormalities, mental def.

A

isotretinoin exposure

33
Q

Gaucher disease

A 
AR LSD
def of glucocerebrosidase enzyme
- subset are asx with painless HSM
- dec plt, anemia
- severe: bone abn: pain crisis, avasc necrosis, osteoporosis, fx, abn modeling w/ widiening of distal femurs
tx: enzyme replacement tx
34
Q

alpha-mannosidosis sx?

A

MR and others

35
Q

Hunter syndrome

A

x-linked MPS
mild or severe
coarse facial features, bone abn

36
Q

Neimann-Pick type A

A

HSM at birth, FTT, neuo deterioration -> death in first few years of life

37
Q

Sanfillippo disease, type B

A

dev delay, coarse features, CNS deterioration

38
Q

risk of mother of kid with DS of having another kid with DS?

A

1% in addition to mom’s age related risk

39
Q

What is the proper course of action after discovering hypospadias in a NB?

A

delay circumcision (to about 6 months) because that is when reocnstruction will be performed

40
Q

Bartter syndrome

A

rare AR; hypokalemia, hypochloremia, high renin and aldosterone

41
Q

Liddle syndrome

A

pseudohyperaldosteronism

  • disorder of renal xport of sodium and potassium
  • infancy/childhood: HTN, polyuria, polydipsia, hypokalemic metabolic acidosis
  • low serum aldosteorn
42
Q

Lesch-Nyhan syndrome

A
  • Xlinked, def of HPRT which catalyzes salvage pathway of purines
  • inc production of uric acid
  • hyperuricemia, uric acid stone, renal impairment, gouty arthritis
  • self-mutilation, choreoathetosis, MR
43
Q

Hunter syndrome

A

MPS II, Xlinkedrecessive, accum of heparan and dermatan sulfate

  • short neck
  • broad chest
  • protub abd
  • HSM
  • umb hernia
  • thoracolumbar kyphosis
  • dec. joint mobility
  • claw fingers
  • stiff gait
  • short stature after age 3
44
Q

What pathogens most commonly affect asplenic pts?

A
  • S. pneu
  • H. flu type B
  • N. meningitidis
45
Q

tx for severe lead poisoning with encephalopathy?

A

EDTA + BAL (dimercaprol)

46
Q

at what lead level do you start tx with a single drug?

A

45; give DMSA (succimer) oral

47
Q

what other conditions can lead to aplastic anemia with parvovirus infxn?

A

SCD
hereditary spherocytosis,
AIHA
(virus halts erythropoeisis in everyone, but pts with rapid RBC turnover will be symptomatic)

48
Q

what does renal U/S do compared to VCUG?

A

U/S looks at urinary tract anatomy/hydropnephrosis;

VCUG is used to dx and evaluate severity of VUR

49
Q

labs indicative of septic arthritis?

A

arthrocentesis: purulent with neutrophil count >50,000 and low glucose conc.

50
Q

when should an infants wt be double the birth wt?

A

6 months

51
Q

when should an infants wt be quadruple the birth wt?

A

24 months

52
Q

when should an infants length be double the birth length?

A

4 years

53
Q

when should an infants wt be triple the birth wt?

A

12 months

54
Q

what is orthostatic proteinuria?

A

total urinary protein excretion rate is higher when child is upright; asx

55
Q

MC IV regimen for CF w/ infxn?

A
  • tobramycin
    AND either antipseudomonal PCN (ticarcillin, piperacillin) OR 3rd gen cephalosporin (ceftazidime or cefepime) OR carbapenem (imipenme/cilastatin or meropenem)
56
Q

What is apnea of prematurity and how is it treated?

A

cessation of air >20 s; assoc w/ dec. HR and hypoxemia

  • happens in half of babies born at 30-31 wks GA, all if <28 wks
  • tx: methylxanthines (theophylline and caffeine) -> stim resp neurons
57
Q

When is epi indicated for neonatal resuscitation?

A

HR <60 bpm despite 100% O2 effective ventilation and chest compression for at least 30 s

58
Q

why is there physiologic steatorrhea in neonates?

A
  • excessive amount of bile acids lost in stools -> poor abs of fat
  • worse in preterm infants
  • tx: substitute medium chain TG for LCTG, because MCTG don’t require bile acids for absorption
59
Q

tx for wiskott aldrich?

A

(triad: dec plt, eczema, rec. infxn)
- splenectomy, continuous abx, IVIG, bone marrow transplant
(inc. risk malignancy - lymphoma, ALL)

60
Q

mass of SCM muscle discovered between 1-8 weeks. dx and management?

A

congenital torticollis aka SCM tumor of infancy

  • usually regress over 4-8 mo
  • conservative tx w/ PT
61
Q

4 year old developmental milestones

A

4 year olds do things in 4s:

  • 4 sided shape (square)
  • count to 4
  • ID 4 colors
  • say 4-5 word sentence
  • picture of a person w/ at least 4 parts
62
Q

at what age can they draw a triangle?

A

5

63
Q

when can 5 digits be repeated?

A

age 7

64
Q

What is the most likely complication of NF1?

A

bony dysplasia - long bone dysplasia, scoliosis, bowing of tibia

65
Q

Above what % is FENa consistent w/ acute RF?

A

> 2%

66
Q

first teeth to erupt in infant?

A

mandibular central incisors

*****aPeds (6 decks)

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