Abstract

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.