Sickle cell disease in gulf cooperation council countries: a systematic review

ABSTRACT

Introduction

Evidence related to the national burden of Sickle Cell Disease (SCD) in Gulf Cooperation Council (GCC) largely fragmented. Thus, the aim of this study is to systemically review studies from GCC countries to assess the epidemiological profile of SCD.

Areas covered

We searched combinations of key terms in MEDLINE/PubMed, CINAHL, and EMBASE. We selected relevant observational studies reporting the frequency, incidence, prevalence, risk factors, mortality rate, and complications of SCD among the GCC population. Studies restricted to laboratory diagnostic tests, experimental and animal studies, review articles, case reports and series, and conference proceedings and editorials were excluded. A total of 1,347 articles were retrieved, out of which 98 articles were found to be eligible and included in the study. The total number of participants from all the included studies was 3,496,447. The prevalence of SCD ranged from 0.24%–5.8% across the GCC and from 1.02%–45.8% for the sickle cell trait. Consanguineous marriage was a risk factor for likely giving children affected with hemoglobinopathies.

Expert opinion

The prevalence of SCD and its complications vary among GCC. Because of the high prevalence of SCD and its complications, health authorities should focus on more rigorous prevention and treatment strategies.

KEYWORDS:

• Sickle cell disease
• epidemiology
• prevalence
• GCC countries
• systematic review

Article highlights

• The prevalence of SCD ranged from 0.24%–5.8% across the GCC.

• The prevalence of SCT ranged between 1.02%–45.8%.

• Hb S and HB SS are the major variants identified in all GCC countries.

• Consanguineous marriage is an important risk factor.

• The prevalence of SCD and its complications are varied among GCC

Acknowledgments

The authors would like to thank the research center at King Fahad Medical City, Riyadh, Saudi Arabia for funding this study.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Author contributions

AS: Carried out the study, participated in the study design, and wrote the final manuscript. HH: Conceived the study, participated in its design and drafted the manuscript. AN and DA: Participated in the study design, in the interpretation of data and drafting the manuscript. DA, AK AND MR: interpretation of data and drafting the article. AA and IA: Participated in the study design, in the interpretation of data and drafting the manuscript. Additionally, all authors read and approved the manuscript.

Availability of data and materials

Data are available upon request from the corresponding author.

Supplementary material

Supplemental data for this article can be accessed online at https://doi.org/10.1080/17474086.2022.2132225

Additional information

Funding

This project was funded under contract no. 019-009 from the Grant Management Department, King Fahad Medical City, Riyadh, Saudi Arabia. The funder did not have any role in the design of the study; the collection, analysis, or interpretation of the data; or the writing of the manuscript.