Systematic Literature Review Shows Gaps in Data on Global Prevalence and Birth Prevalence of Sickle Cell Disease and Sickle Cell Trait: Call for Action to Scale Up and Harmonize Data Collection

Abstract

Sickle cell disease (SCD) is an inherited monogenic disorder with high prevalence throughout sub-Saharan Africa, the Mediterranean basin, the Middle East, and India. Sources of SCD epidemiology remain scarce and fragmented. A systematic literature review (SLR) to identify peer-reviewed studies on SCD epidemiology was performed, with a search of bibliographic databases and key conference proceedings from 1 January 2010 to 25 March 2022 (congress abstracts after 2018). The SLR followed PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. Meta-analyses, using a binomial normal random-effects model, were performed to estimate global and regional prevalence and birth prevalence. Of 1770 journal articles and 468 abstracts screened, 115 publications met the inclusion criteria. Prevalence was highest in Africa (~800/100,000), followed by the Middle East (~200/100,000) and India (~100/100,000), in contrast to ~30/100,000 in Europe. Birth prevalence was highest in Africa (~1000/100,000) and lowest in North America (~50/100,000) and Europe (~30/100,000). This SLR confirmed that sub-Saharan and North-East Africa, India, the Middle East, and the Caribbean islands are global SCD hotspots. Publications including mortality data were sparse, and no conclusions could be drawn about mortality. The identified data were limited due to gaps in the published literature for large parts of the world population; the inconsistent reporting of SCD genotypes, diagnostic criteria, and settings; and a sparsity of peer-reviewed publications from countries with assumed high prevalence. This SLR demonstrated a lack of systematic knowledge and a need to provide uniform data collection on SCD prevalence and mortality.

Malnutrition in sickle cell anemia: Prevalence, impact, and interventions: A Review

“…These tools provide a comprehensive evaluation, considering disease-specific factors such as medication usage, dietary restrictions, and the impact of SCD on nutritional intake. [69] 12. Future directions of malnutrition in SCA The management of malnutrition in individuals with SCA is an evolving field with ongoing research and potential future directions.…”

“…Various nutrition screening tools have been developed to assess the specific needs and challenges associated with SCD. [ 69 ] One such tool is the Malnutrition Universal Screening Tool (MUST), which is commonly used in diverse populations but may need adaptation for the unique considerations of SCA. [ 70 ] Malnutrition Universal Screening Tool (MUST), or any similar tool, can serve as a valuable asset in identifying individuals at risk of malnutrition in the context of SCD.…”

“…These tools provide a comprehensive evaluation, considering disease-specific factors such as medication usage, dietary restrictions, and the impact of SCD on nutritional intake. [ 69 ]…”

Correction: Colombatti et al. Systematic Literature Review Shows Gaps in Data on Global Prevalence and Birth Prevalence of Sickle Cell Disease and Sickle Cell Trait: Call for Action to Scale Up and Harmonize Data Collection. J. Clin. Med. 2023, 12, 5538

“…In the original publication [1], there was a mistake in Table 1. The total number of studies and the total study population for the prevalence of SCD in Europe, the global birth prevalence and the global prevalence of SCT per 100,000 were amended.…”

Sickle Cell Disease Update: New Treatments and Challenging Nutritional Interventions

“…Despite the increase in the global burden of SCD, which is believed to affect over 20 million people [ 32 ], including an estimated 200,000 annual sickle genotype births in Sub-Saharan Africa [ 33 ], available data on SCA prevalence, morbidity, and mortality remains limited on a global scale. However, some systematic reviews on global data exist [ 34 , 35 ].…”