Pediatrics Flash Cards - Case 30 - SCD

Question 1

What are the four most common genotypes of sickle cell disease seen in the U.S. ?

Answer 1

HbSS (65% cases) and Sβ(o)-thalassemia (2% cases) = more severe than HbSC (25% cases) or Sβ(+) thalassemia (8% cases)

Question 2

Presentation of young child w/ SCD who was not screened?

Answer 2

dactylitis, pneumococcal sepsis or meningitis, severe anemia and acute splenic enlargement, acute chest syndrome, pallor, or jaundice

Question 3

Presentation of older child/adolescent w/ SCD who was not screened?

Answer 3

anemia, severe or recurrent MSK or abd pain, aplastic crisis, acute chest syndrome, priapsim, splenomegaly/sequestration, and cholelithiasis

Question 4

What is dactylitis?

Answer 4

painful swelling of the hands and feet

Question 5

SCD patients of what ages get prophylaxis? What drug? Dose? If allergic?

Answer 5

Pts w/ HbSS and Sβ(o)-thalassemia: 2mo to 35mo (Pen VK 125mg BID) 3yr to 5 yr (Pen VK 250 BID); erythromycin if allergic

Question 6

Two most common surgeries in sickle cell kids?

Answer 6

Cholecystectomy and Tonsillectomy

Question 7

To which type of organisms are asplenic susceptible? ¿Ejemplos?

Answer 7

encapsulated bacteria: Strep pneu, Hib, Neisseria meningitidis

Question 8

By what age may some SCD kiddos develop splenic insufficiency?

Answer 8

3 m/o

Question 9

ROS questions for parent of a SCD at a quarterly health maintencen visit?

Answer 9

How often pain? How well controlled? ED? Inpt? How often fever…? Transfusion hx? When do you know to call/bring in? How copinh?

Question 10

How common is stroke in SCD kids?

Answer 10

Up to 10% of SCD pts will have a stroke by 15 y/o.

Question 11

What is the inheritance pattern of SCD?

Answer 11

autosomal recessive

Question 12

What is typical range of baseline Hgb in SCD kiddos?

Answer 12

Children with sickle cell disease frequently have baseline hemoglobins between 6 and 9 g/dL

Question 13

DDx for sudden onset chest pain in kiddo w/ SCD?

Answer 13

acute chest syndrome, CHF, rib infarction, sepsis, pericarditis

Question 14

What are the clinical features of acute chest syndrome?

Answer 14

Fever (80%), Cough (62%), CP (40%) [can lead to -> difficulty w/ expansion of lwr lung -> atelectasis], SOB, Decr oxygenation

Question 15

What are the clinical features of rib infarction?

Answer 15

Normal O2 sat (vs ACS); Usu nl CXR though may lead to an ACS picture as pain -> hypoventilation -> atelectasis and characteristic radiographic findings of ACS

Question 16

Prevnar vs. Pneumovax?

Answer 16

Prevnar is the routine PCV7 given over 4 doses before age 2; Pneumovax is PPSV23 given at 2 again 5-8 y/o to kids at high risk for invasive pneumococcal infection (sickle cells, asplenia, cochlear implants, CSF leaks, HIV, immunocompromise, chronic heart/lung dz, or immunosuppressive meds)