Value Added Abstract
Amani Abu-Shaheen
Abstract
A Sickle cell disease (SCD) is an autosomal recessive disorder characterized by the production of abnormal hemoglobin S. The incidence of SCD in the Arabian Peninsula ranged from 1.2 to 2.6%. In Saudi Arabia, the prevalence of SCD varies significantly in different parts of the country, with the highest incidence reported in the Eastern province. The mortality rates associated with SCD vary considerably worldwide. Countries in the Gulf region have adopted different preventive measures over the years, like newborn screening programs, a premarital, and counseling program. Various factors have contributed to inadequate data on the real magnitude of health and economic burden of SCD in the Gulf Cooperation Council (GCC) countries. The national burden of SCD in Saudi Arabia is reported in fragmented scientific works. Moreover, although screening tests are available in major hospitals, genetic counseling services are few and far between which, together with low genetic literacy among the public, results in a lack of awareness about genetic disorders and possibilities for the prevention of these disorders. Objectives: We aimed to systemically review the published studies to identify the epidemiological profile of SCD in the GCC countries. Methods: We systemically searched MEDLINE/PubMed, CINAHL, and EMBASE and selected relevant studies reporting the epidemiology of SCD among the GCC population. Data on the incidence, prevalence, risk factors, mortality rate, and complications of SCD were extracted. We assessed the quality of the retrieved studies according to the Newcastle–Ottawa Quality Assessment Scale developed for cohort studies and the modified version designed for cross-sectional studies.