The prevalence of sickle cell disease in Saudi children and adolescents. A community-based survey

OBJECTIVE: To determine the prevalence and regional distribution of sickle cell disease in Saudi children.

METHODS: A sample size of 45,682 children and adolescents from newborn to 19 years of age was selected by multistage random probability sampling of the Saudi households from each of the 13 regions of the country. The study is cross-sectional, community based, and conducted over 2 years from 2004 to 2005. Data, including history and clinical examination were collected with house-to-house survey of all selected households. Data management and analysis was carried out at King Saud University, Riyadh, Saudi Arabia.

RESULTS: Sickle cell disease was detected in 108 of 45,682 children and adolescents with a prevalence of 24 per 10,000. The regional distribution of sickle cell disease showed eastern region dominance with a prevalence of 145 per 10,000, followed by the southern region with a prevalence of 24 per 10,000, western region 12 per 10,000, and central region with 6 per 10,000. No cases were found in the northern regions. The male to female ratio was approximately 1:1.

CONCLUSION: The results of this national wide community-based survey show a high prevalence of sickle cell disease in the community and the disease is more common in eastern and southern regions of the country. National or regional newborn screening programs for sickle cell disease using hematological tests should be planed. This study shows that the population at risk has an uneven geographical distribution. For this reason, selective rather than universal neonatal screening is likely to be more appropriate in the country.