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Clinical profile of sickle cell disease in Yemeni children
Abdul-Wahab Al-Saqladi1, Ali Delpisheh, Hassan Bin-Gadeem
1Faculty of Medicine & Health Sciences, Aden University, Yemen.
Insights
Sickle cell disease (SCD) significantly impacts Yemeni children, with early onset and common symptoms like dactylitis. Management requires screening, comprehensive care, and genetic counseling for better outcomes.
Area of Science:
- Pediatrics
- Hematology
- Genetics
Background:
- Sickle cell disease (SCD) presents a diverse clinical spectrum across the Arabian Peninsula.
- This study addresses the first report on SCD in Yemeni children.
Purpose of the Study:
- To describe the clinical manifestations and early-onset characteristics of sickle cell disease (SCD) in Yemeni children.
- To compare the disease course and severity in Yemeni children with existing data from other populations.
Main Methods:
- A hospital-based, cross-sectional study was conducted.
- Included were children under 16 years diagnosed with homozygous sickle cell disease (SS SCD).
- Data collected at Al-Wahada Teaching Hospital in Aden.
Main Results:
- Clinical manifestations were observed in 20% by 6 months and up to 92% by 3 years.
- Dactylitis (hand-foot syndrome) was the most frequent presenting symptom (54%), followed by infections.
- Hepatomegaly (72%) and splenomegaly (40%) were common; painful crisis (36%) led to hospitalization.
Conclusions:
- Sickle cell disease (SCD) is a severe pediatric health issue in Yemen, manifesting early in life.
- The disease course and severity in Yemeni children resemble those in African and American black populations.
- A comprehensive screening program, medical care, and genetic counseling are crucial for improving management and quality of life.
Unlabelled:
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.
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