A 1-year-old African American girl is brought to the office for routine evaluation. She is learning how to walk and says "mama" and "dada." She recently transitioned from breast milk to cow's milk. Review of systems is negative. Both parents have sickle cell trait and her sister has sickle cell disease. Her height and weight are appropriate for her age. Physical examination shows a well-appearing, well-nourished child with no abnormalities. Laboratory results are as follows: What is the most common complication of her condition?

Question

A 1-year-old African American girl is brought to the office for routine evaluation.  She is learning how to walk and says "mama" and "dada."  She recently transitioned from breast milk to cow's milk.  Review of systems is negative.  Both parents have sickle cell trait and her sister has sickle cell disease.  Her height and weight are appropriate for her age.  Physical examination shows a well-appearing, well-nourished child with no abnormalities.  Laboratory results are as follows:  What is the most common complication of her condition?

Quizplus · Accepted Answer

11ec25c8_d6ab_c1ea_bc81_9bc83b8bb6e9_MD0003_00 Sickle cell disease is a hemoglobinopathy with an autosomal recessive inheritance. When both parents carry the trait, their children are at risk for inheriting the disease. This patient's hemoglobin electrophoresis pattern and normal complete blood count are consistent with being a carrier for the sickle cell trait. Individuals with sickle cell trait are generally asymptomatic and can lead a healthy life. Although patients are at increased risk for renal issues, the most common of these is painless microscopic or gross hematuria that results from sickling in the renal medulla. Hyposthenuria (impairment in concentrating ability) is also common and can present as nocturia and polyuria. Less commonly, there may be an increased risk of urinary tract infections, particularly during pregnancy (Choice G). Splenic infarctions (Choice F) are uncommon but can occur at high altitudes. (Choices A, B, D, and E) Other complications of sickle cell disease are unlikely to occur with sickle cell trait. Educational objective: Most patients with sickle cell trait lead normal, healthy lives. Painless hematuria is the most common complication. References: Complications associated with sickle cell trait: a brief narrative review. (http://www.ncbi.nlm.nih.gov/pubmed/19393983) Renal abnormalities in sickle cell disease. (http://www.ncbi.nlm.nih.gov/pubmed/10620181)

A 1-Year-Old African American Girl Is Brought to the Office