Clinical profile of sickle cell disease in Yemeni children

Al-Saqladi, A. W., Delpisheh, Ali, Bin-Gadeem, H. and Brabin, Bernard (2007) 'Clinical profile of sickle cell disease in Yemeni children'. Annals of Tropical Paediatrics, Vol 27, Issue 4, pp. 253-259.

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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.

Item Type:	Article
Uncontrolled Keywords:	natural-history saudi-arabia anemia gene hemoglobin prevalence management enuresis
Subjects:	WB Practice of Medicine > Medical Climatology > WB 710 Diseases of geographic areas WH Hemic and Lymphatic Systems > Hematologic Diseases. Immunologic Factors. Blood Banks > WH 170 Hemolytic anemia (e.g., Sickle cell anemia) WS Pediatrics > Diseases of Children and Adolescents > By System > WS 300 Hemic and lymphatic system
Faculty: Department:	Groups (2002 - 2012) > Child & Reproductive Health Group
Digital Object Identifer (DOI):	https://doi.org/10.1179/146532807X245634
Depositing User:	Ms Julia Martin
Date Deposited:	31 Aug 2010 15:00
Last Modified:	06 Feb 2018 13:01
URI:	https://archive.lstmed.ac.uk/id/eprint/1159

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