High fetal hemoglobin level is associated with increased risk of cerebral vasculopathy in children with sickle cell disease in Mayotte.

ABSTRACT:

Background

Understanding the genetics underlying the heritable subphenotypes of sickle cell anemia, specific to each population, would be prognostically useful and could inform personalized therapeutics.The objective of this study was to describe the genetic modulators of sickle cell disease in a cohort of pediatric patients followed up in Mayotte.

Methods

This retrospective cohort study analyzed clinical and biological data, collected between January1^st2007 and December 31^st2017, in children younger than 18 years.

Results

We included 185 children with 72% SS, 16% Sβ0-thalassemia and 12% Sβ + thalassemia. The average age was 9.5 years; 10% of patients were lost to follow up. The Bantu haplotype was associated with an increase in hospitalizations

SUBMITTER: Chamouine A 

PROVIDER: S-EPMC7305627 | biostudies-literature | 2020 Jun

REPOSITORIES: biostudies-literature