UWorld Flashcards
S/sx of tetralogy of fallot
Cyanosis
Harsh systolic ejection murmur left sternal border due to RV outflow obstruction
Squatting increases after load and decreases right-to-left shunting, helping end tet spells
Squatting increases preload and thus murmur due to inc flow across RVOT obstruction
How does blood flow in a VSD? And in TOF?
Normal VSD is a left to right shunt due to lower pressure on right side, and are usually not cyanotic.
VSD in Tet becomes right to left due to RVOT obstruction, and RV hypertrophy.
Squatting in VSD?
Increased SVR increases murmur by directing blood through to right side
How does hypertrophic cardiomyopathy murmur change with squatting? Handgrip?
Squatting and handgrip increase SVR = decreased blood flow through LVOT, and decreased murmur.
Sickle cell anemia - 3 signs, 1 consequence, 2 prophylaxis
Anemia
High reticulocyte count
History of pain crises
Functional hyposplenia - increased susceptibility to encapsulated organisms
Twice daily penicillin prophylaxis until age 5
Vaccination with conjugate capsular polysaccharide
What are nevus simplex lesions? What should be done with them?
Stork bites or angel kisses
Normally resolve by 12 months of age
What should be done with superficial hemangiomas?
May appear in first two weeks of life and grow rapidly for two years. Most are harmless and regress spontaneously during childhood. If they are in a problematic location or ulcerating, they may be treated with beta blockers
What should be done if a child swallows a battery?
X-ray to determine location of the battery. If the battery is in the esophagus, immediate endoscopic removal is indicated. If it has passed beyond the esophagus, 90% will pass uneventfully. Observation to confirm passage by stool examination or radiographic follow up.
How does sickle cell disease differ from sickle cell trait on hemoglobin electrophoresis?
Disease = Hemoglobin S 85-95% Hemoglobin A 0% Hemoglobin F 5-15% Trait = Hemoglobin S 35-45% Hemoglobin A 50-60% Hemoglobin F less than 2%
What is the most common complication of sickle cell trait?
Painless Hematuria, either microscopic or gross
Isothenuria (impairment in concentrating ability) is also common and may cause nocturia or polyuria.
Path and Clinical findings in Henoch-Schonlein purpura
Path: IgA mediated leukocytoclastic vasculitis Palpable purpura Arthritis/Arthralgia Abdominal pain, intussusception Renal disease similar to IgA nephropathy
Lab and kidney findings of Henoch-Schonlein purpura
Normal platelet count and coag studies
Normal to increased creatinine
Hematuria w/wo RBC casts w/wo proteinuria
Kidneys: Mesangial deposition of IgA
Treatment for Henoch-Scholein purpura
Supportive (Hydration and NSAIDS) for most patient
Hospitalization and systemic steroids in patients with severe symptoms
What is the most common type of idiopathic nephrotic syndrome in children, what does it present with, and what are the EM findings?
Minimal change disease
Presents with edema and hematuria
EM shows fusion or flattening of the podocytes
Crigler-Najjar vs Gilberts
Both inherited deficiencies of UDP-glucuronyl transferase
Both result in unconjugated hyperbilirubinemia
Gilberts is mild with period jaundice is times of stress
C-N is total absence of the enzyme and requires liver transplant
Rash in lyme disease
Erythematous patch with central area of pallor
May be expanding slowly
No itch or pain
No associated symptoms
What features do homocystinuria and marfan syndrome share?
Pectus deformity Tall stature (inc arm:height ratio, dec upper:lower segment ratio) Arachnodactyly Joint hyper laxity Skin hyper elasticity Scoliosis
What features does Marfan’s syndrome have that homocystinuria does not?
Marfan: Aortic root dilation, normal intellect, upward lens dislocation, autosomal dominant
What features does homocystinuria have that Marfan’s does not?
Intellectual disability Thrombosis (strokes, etc.) Downward lens dislocation Megaloblastic anemia Fair complexion Autosomal recessive
How is Ehler’s-Danlos syndrome different from homocystinuria?
ED does not have tall stature, lens dislocation, or hyper coagulability.
ED does have scoliosis, hyper extensible joints and hyper elastic skin, like homocystinuria and Marfan’s
Clinical signs of iron poisoning?
30 min to 4 days: Abdominal pain, vomiting (hematemesis, corrosive), diarrhea (melena), anion-gap metabolic acidosis, hypotensive shock
2 days: Hepatic necrosis
2-8 weeks: Pyloric stenosis
Dx and treatment of iron poisoning?
Dx: Anion gap acidosis, Radiopaque pills on x-ray
Tx: Whole bowel irrigation, deferoxamine, Supportive care for A, B, Cs
S/sx of Vitamin A overdose?
Nausea, vomiting, blurry vision. Chronically leads to pseudo tumor cerebri.
S/sx of Vit D overdose?
Related to hypercalcemia including nausea, vomiting, confusion, polyuria, polydipsia.
