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Congenital Heart > UWorld 2 > Flashcards

UWorld 2 Flashcards

1
Q

What are symptoms of vaso-occlusion in sickle cell anemia?

A
  • Hand foot syndrome / dactylitis - swelling of the hands/feet, w/ restricted ROM
  • presents at age 6 mo – 2 yrs
  • acute onset, symmetric pain/swelling
  • sometimes fever
  • vascular necrosis of the metacarpals/metatarsals
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2
Q

What is the molecular basis of sickle cell disease?

A

Point mutation: substitution of valine for glutamic acid

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3
Q

HSP (henoch-schloen purpura)

A
  • most common systemic vasculitis of childhood
  • follows minor infxn
  • tetrad: palpable purpura of the LE, arthralgia (ankles/knees), abdominal pain, renal disease (hematuria, non-nephrotic range proteinuria, elevated serum creatinine, IgA deposition of the mesangium)
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4
Q

What is the distinguishing feature of HSP?

A

-nL platelet count

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5
Q

Purpura fulminans

A

seen w/ bacterial infxns (n. meningitides, strep pneumo) – blue/black hemorrhagic purpuric lesions
-fever, hypotension, DIC

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6
Q

What viral exanthems might cause purpura?

A

Rocky mountain spotted fever / atypical measles

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7
Q

Triad of Kartegener’s syndrome:

A

recurrent sinusitis, bronchiectasis, dextrocardia
-primary ciliary dyskinesia due to aberrant production/attachment of dynein arms
Autosomal recessive + cilia dysmotility

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8
Q

What are the subtypes of ADHD?

A

Predominantly inattentive, predominantly hyperactive/impulsive; combined type

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9
Q

Diagnostic criteria for ADHD in kids?

A
  • symptom onset before age 12
  • > 2 settings
  • functional impairment
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10
Q

What is the treatment of kawasaki’s disease

A

Aspirin + IV Ig w/in 10 days of fever onset

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11
Q

What are complications of IV Ig in untreated patients?

A

> 20% of pts à MI/death

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12
Q

How to monitor or F/U Kawasaki?

A

-echocardiogram + repeat 6-8 wks later

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13
Q

What is Reye’s syndrome?

A

-life-threatening hepatic encephalopathy

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14
Q

What is the rash of scarlet fever like? What are other findings?

A

Presents following untreated streptococcal pharyngitis. Lacks tonsillar exudates, sandpaper scarlet fever rash that spares palms/soles

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15
Q

How to evaluate scarlet fever?

A

Amoxicillin / throat culture

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16
Q

What is presentation of rocky mountain spotted fever?

A

Rash on palms / soles; GI symptoms; headache;

treated w/ doxycycline for 5-7 days

17
Q

How to f/u on a white-reflex discovered on an eye exam?

A

Leukocoria = retinoblastoma until proven otherwise
Requires prompt referral to opthal
Inactivation of Rb suppressor gene – may be familial / sporadic

18
Q

What is the most prevalent cardiac anomalies in Down syndrome?

A

50% of pts w/ cardiac disease

Complete atrioventricular septal defect most common, next = VSD + ASD

19
Q

What is heard on CAVSD?

A
  • loud S2 (pulm HTN)
  • systolic ejection murmur (incr. flow across pulm valve from L à R shunt)
  • holosystolic murmur (soft/absent if defect is large)
20
Q

What are characteristic findings of ewing’s sarcoma?

A
  • malignant tumor
  • most commonly metaphysic / diaphysis of femur, tibia, humerus (in that order)
  • pain/swelling, leukocytosis, anemia, incr. ESR
  • lamellated/onion skin periosteal rxn
  • central lytic lesion + endosteal scalloping
21
Q

What is brodie’s abscess?

A

-chronic osteomyelitis w/ central lytic bone defect with surrounding sclerosis

22
Q

What is Legg-Calve-Perthes disease?

A

-idiopathic osteonecrosis / avascular necrosis of the femoral head

23
Q

How does slipped capital femoral epiphysis present? And in what demographic?

A

Limp + insidious hip pain

-mean age of presentation = girls at age 12, boys at age 13.5

24
Q

Intussussception

A

invagination of the ileum into the colon of the ileocecal valve
periodict irritability, colic, emesis

25
Q

hirschsprung clues

A
  • failure to pass meconium w/in first 24-48 hrs
  • repeated rectal stimulation to induce BM
  • poor feeding/bilious vomiting, abdominal distension (1st mo of life)
26
Q

pyloric stenosis

A

-gastric outlet obstruction / emesis 1st 2 mo of life

27
Q

TCA overdose treatment?

A
  • sodium bicarb - cardiac toxicity (prolonged QRS > 100 msec) + ventricular arrhythmias (v. tachy / v. fib)
  • benzos: seizures in overdose
28
Q

how to treat cat bites prophylactically? what are concerning signs?

A
  • pathogeN: pasturella multocida
  • cellulitis signs: pain, erythema, swelling, lymphadenopathy
  • prophylactic treatment: 5 days amoxicillin-clavulanate
29
Q

First line treatment for urinary continence

A

if > 5 yo & have attempted lifestyle
1st line = desmopressin
2nd line = TCA

30
Q

monosymptomatic enuresis

A

urinary incontinence >2x / wk after age 5

Congenital Heart (12 decks)

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