UWorld 2 Flashcards
What are symptoms of vaso-occlusion in sickle cell anemia?
- 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
What is the molecular basis of sickle cell disease?
Point mutation: substitution of valine for glutamic acid
HSP (henoch-schloen purpura)
- 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)
What is the distinguishing feature of HSP?
-nL platelet count
Purpura fulminans
seen w/ bacterial infxns (n. meningitides, strep pneumo) – blue/black hemorrhagic purpuric lesions
-fever, hypotension, DIC
What viral exanthems might cause purpura?
Rocky mountain spotted fever / atypical measles
Triad of Kartegener’s syndrome:
recurrent sinusitis, bronchiectasis, dextrocardia
-primary ciliary dyskinesia due to aberrant production/attachment of dynein arms
Autosomal recessive + cilia dysmotility
What are the subtypes of ADHD?
Predominantly inattentive, predominantly hyperactive/impulsive; combined type
Diagnostic criteria for ADHD in kids?
- symptom onset before age 12
- > 2 settings
- functional impairment
What is the treatment of kawasaki’s disease
Aspirin + IV Ig w/in 10 days of fever onset
What are complications of IV Ig in untreated patients?
> 20% of pts à MI/death
How to monitor or F/U Kawasaki?
-echocardiogram + repeat 6-8 wks later
What is Reye’s syndrome?
-life-threatening hepatic encephalopathy
What is the rash of scarlet fever like? What are other findings?
Presents following untreated streptococcal pharyngitis. Lacks tonsillar exudates, sandpaper scarlet fever rash that spares palms/soles
How to evaluate scarlet fever?
Amoxicillin / throat culture
What is presentation of rocky mountain spotted fever?
Rash on palms / soles; GI symptoms; headache;
treated w/ doxycycline for 5-7 days
How to f/u on a white-reflex discovered on an eye exam?
Leukocoria = retinoblastoma until proven otherwise
Requires prompt referral to opthal
Inactivation of Rb suppressor gene – may be familial / sporadic
What is the most prevalent cardiac anomalies in Down syndrome?
50% of pts w/ cardiac disease
Complete atrioventricular septal defect most common, next = VSD + ASD
What is heard on CAVSD?
- 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)
What are characteristic findings of ewing’s sarcoma?
- 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
What is brodie’s abscess?
-chronic osteomyelitis w/ central lytic bone defect with surrounding sclerosis
What is Legg-Calve-Perthes disease?
-idiopathic osteonecrosis / avascular necrosis of the femoral head
How does slipped capital femoral epiphysis present? And in what demographic?
Limp + insidious hip pain
-mean age of presentation = girls at age 12, boys at age 13.5
Intussussception
invagination of the ileum into the colon of the ileocecal valve
periodict irritability, colic, emesis
