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.


CONCLUSIONS
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.
Faculty of Medicine & Health Sciences, Aden University, Yemen.
Abdul-Wahab
AW
Al-Saqladi
Ali
A
Delpisheh
Hassan
H
Bin-Gadeem
Bernard J
BJ
Brabin
D000293:Adolescent; D017677:Age Distribution; D000755:Anemia, Sickle Cell / Q000150:complications / Q000208:ethnology*; D001157:Arterial Occlusive Diseases / Q000208:ethnology / Q000209:etiology; D002637:Chest Pain / Q000208:ethnology / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D005260:Female; D005385:Fingers / Q000098:blood supply; D006760:Hospitalization; D006801:Humans; D007223:Infant; D008297:Male; D009894:Opportunistic Infections / Q000150:complications / Q000208:ethnology; D012141:Respiratory Tract Infections / Q000150:complications / Q000208:ethnology; D017678:Sex Distribution; D014034:Toes / Q000098:blood supply; D015006:Yemen / Q000453:epidemiology