Clinical profile of sickle cell disease in Yemeni children

The clinical spectrum of sickle cell disease (SCD) in the Arabian Peninsula varies widely. This is the first report in Yemeni children.

Methods: A hospital-based, cross-sectional study was undertaken in Al-Wahada Teaching Hospital in Aden of children under 16 years with homozygous (SS) SCD.

Results: Fifty-six (55%) were males. There were clinical manifestations in 20% by the age of 6 months and in 67%, 88% and 92% by 1, 2 and 3 years, respectively. Dactylitis (hand-foot syndrome) was the most common presenting symptom and occurred in 54% of cases, followed by acute respiratory infections and other acute febrile illnesses. The main causes of hospitalisation were painful crisis (36%), anaemic crisis (16%) and acute chest syndrome (11%). Hepatomegaly was detected in 72% and splenomegaly in 40%. Cerebrovascular accident, cholelithiasis, hepatic crisis and leg ulcers each occurred in about 5% of patients. There was first-and second-degree consanguinity in 31% and 16%, respectively, of patients' families.

Conclusion: SCD is a serious problem, affecting children in Yemen from an early age. Disease course and severity were similar to that in Africans and American blacks and some reports from western Saudi Arabia. A screening programme linked to comprehensive medical care and genetic counselling is required to improve management and quality of life.