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Epidemiology of Sickle Cell Disease in Gulf Cooperation Council Countries: A Systematic Review

88 Pages Posted: 3 Aug 2019

See all articles by Amani Abu-Shaheen

Amani Abu-Shaheen

King Fahad Medical City - Research Center

Humariya Munshi

King Fahad Medical City - Research Center

Abdullah Nofal

King Saud University - College of Medicine

Doaa A. Abdelmoety

King Abdullah Medical City

Muhammad Riaz

University of Malakand

Isamme AlFayyad

King Fahad Medical City - Research Center

More...

Abstract

Objectives: We aimed to systemically review the published studies to assess the epidemiological profile of sickle cell disease (SCD) in the Gulf Cooperation Council (GCC) countries.

Methods: We systemically searched MEDLINE/PubMed (National Library of Medicine), CINAHL, and EMBASE through December 2018 and selected relevant studies reporting the epidemiology of SCD among GCC population. Data of 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.

Results: Fifty-one studies were included in our systematic review. The prevalence rates of SCD varied from 0.2 to 21%. The most frequent risk factor was consanguineous marriage. The most common SCD-associated complications reported were acute chest syndrome, hemolytic crisis, pain crisis, infections, vaso-occlusive crisis, and splenic complications. The incidence rates of hemolytic crisis ranged from 4.46% to 100%. The highest incidence rate of pain crisis was reported in Alsultan et al., 2012 (96%). The splenic complications were in the form of acute splenic sequestration and splenomegaly or hypersplenism. The rates of acute chest syndrome ranged from 1% up to 100%. Various types of infections, such as upper and lower respiratory tract infections, gastroenteritis, septicemia, osteomyelitis, or septic arthritis, were reported. The highest incidence rate of infection was 1989 (63.80%). The mortality rate ranged between 0.4% and 28.7%. The incidence rate of the vaso-occlusive crisis ranged between 21% and 98%; the highest incidence rate was of more than 95%.

Conclusions: We recommend additional promotion in preventive measures in the high-risk provinces and intensify the community perception to implement a rate reduction for these disorders.

Funding Statement: This project was funded under contract no. 019-009 from the Grant Management Department, King Fahad Medical City. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.

Declaration of Interests: The authors have declared that no competing interests exist.

Ethics Approval Statement: The authors declare they followed the PRISMA checklist when reporting this manuscript and all steps were done in strict compliance with Cochrane handbook of systematic reviews for interventions.

Keywords: Sickle cell disease; Epidemiology; Prevalence; GCC countries; Systematic review

Suggested Citation

Abu-Shaheen, Amani and Munshi, Humariya and Nofal, Abdullah and Abdelmoety, Doaa A. and Riaz, Muhammad and AlFayyad, Isamme, Epidemiology of Sickle Cell Disease in Gulf Cooperation Council Countries: A Systematic Review (July 31, 2019). Available at SSRN: https://ssrn.com/abstract=3429912 or http://dx.doi.org/10.2139/ssrn.3429912

Amani Abu-Shaheen (Contact Author)

King Fahad Medical City - Research Center ( email )

Saudi Arabia

Humariya Munshi

King Fahad Medical City - Research Center

Saudi Arabia

Abdullah Nofal

King Saud University - College of Medicine

Saudi Arabia

Doaa A. Abdelmoety

King Abdullah Medical City

Saudi Arabia

Muhammad Riaz

University of Malakand

Chakdara Dir (lower)
N.W.F.P 42000
Pakistan

Isamme AlFayyad

King Fahad Medical City - Research Center ( email )

Saudi Arabia

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