A 2-year-old African-American child presents with painful swelling of the hands and feet.
Laboratory evaluation reveals hemoglobin of 9 g/dL with white blood cell count of 11,500
and platelet count of 250,000. Which additional laboratory test will support your diagnosis?
(A) skeletal survey
(B) VDRL testing
(C) bone marrow aspiration
(D) hemoglobin electrophoresis
(E) serum calcium measurement
Answer :
(D) The child described has the classic handfoot syndrome seen in infants and toddlers with sickle cell disease. Dactylitis, presumably secondary to infarction of the small bones, causes painful swelling of the hands and feet. Hemoglobin electrophoresis would show presence of high levels of HbS.
In children younger than 5 years, the small bones of the hands and feet are frequently affected, and in contrast to most episodes of bone pain in older children, physical findings are common.
This painful dactylitis (“hand-foot syndrome”) is typically the first clinical manifestation of SCD. A young child cries with pain; refuses to bear weight; and has puffy, tender, and warm feet or hands, or both. The child may appear acutely ill, be febrile, and have an impressive leukocytosis. At the onset of soft tissue swelling, bony changes are not generally apparent on radiographs.
After 1 to 2 weeks, subperiosteal new bone, irregular areas of radiolucency, cortical thinning, or complete destruction of bone can be seen. All the bone changes are usually reversible but may persist for as long
as 8 months. A rare complication, permanent shortening of the digits after hand-foot crisis, has been
reported. Dactylitis before 1 year of age is a strong predictor of overall severity (stroke, death, high pain rate, or recurrent ACS) by 10 years of age, although recent single-institution evidence suggests that dactylitis is not a strong predictor of subsequent pain or ACS.
ليست هناك تعليقات:
إرسال تعليق