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Prévalence du variant Q248H du gène SLC40A1 codant la ferroportine dans la population de patients drépanocytaires présentant une surcharge en fer
Mémoire de Master / Thèse d'exercice   Open Access

Prévalence du variant Q248H du gène SLC40A1 codant la ferroportine dans la population de patients drépanocytaires présentant une surcharge en fer

Romain Baury
Masters , Université de Montpellier
17/10/2023

Résumé

Sickle cell disease Iron overload African hemosiderosis Ferroportin Q248H variant Syndrome drépanocytaire majeur Ferroportine Variant Q248H SLC40A1 Surcharge en fer Drépanocytose Hémosidérose Hémochromatoses
Iron overload in sickle cell disease is a common issue and is responsible for increased morbidity and mortality. It primarily occurs in a post-transfusion context but not exclusively. Indeed, among the risk factors of iron overload, there are likely genetic predispositions. In France, the majority of sickle cell disease have African origin. In this population, there is a phenotype of iron overload known as African hemosiderosis. It is multifactorial origin and associated with the presence of the Q248H variant of the SLC40A1 gene coding for ferroportin. We hypothesized that this variant is significantly over-represented in iron-overloaded sickle cell disease patients. This study presents the retrospective data collection of a sickle cell disease population with iron overload follow at the Montpellier University Hospital and the prospective search of the Q248H variant.

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