title: Comparative Analysis of Iron Homeostasis in Sub-Saharan African Children with Sickle Cell Disease and Their Unaffected Siblings.
abstract: Iron is an essential trace element subject to tight regulation to ensure adequate running of biological processes. In sub-Saharan Africa where hemoglobinopathies are common, iron homeostasis is likely to be impaired by these conditions. Here, we assessed and compared key serum proteins associated with iron metabolism between sub-Saharan African children with sickle cell disease (SCD) and their unaffected siblings. Complete blood counts and serum concentrations of four key proteins involved in iron regulation (ferritin, transferrin, sTfR, and hepcidin) were measured for 73 children with SCD and 68 healthy siblings in Benin, West Africa. We found significant differences in concentration of transferrin, sTfR, and ferritin between the two groups. Hepcidin concentrations were found at unusually high concentrations but did not differ among the two groups. We found a significant negative correlation between hepcidin levels and both MCH and MCV in the SCD group and report that sTfR concentrations show a correlation with MCV and MHC in opposite directions in the two groups. These results highlight the unusually high levels of hepcidin in the Beninese population and the patterns of differential iron homeostasis taking place under SCD status. These results lay the foundation for a systematic evaluation of the underlying mechanisms deregulating iron homeostasis in populations with SCD or high prevalence of iron deficiency.
authors.affiliation: Laboratoire de Biochimie et de Biologie Moléculaire, Faculté des Sciences et Techniques, University of Abomey-Calavi, Cotonou, Benin; Centre de Prise en charge Médicale Intégrée du Nourrisson et de la Femme Enceinte atteints de Drépanocytose, Faculté des Sciences de la Santé, University of Abomey-Calavi, Cotonou, Benin.
authors.forename: Selma
authors.initials: S
authors.lastname: Gomez
authors.affiliation: Biology Program, Division of Science and Mathematics, New York University Abu Dhabi , Abu Dhabi , United Arab Emirates.
authors.forename: Aïssatou
authors.initials: A
authors.lastname: Diawara
authors.affiliation: Sainte-Justine Research Centre, Centre Hospitalier et Universitaire Sainte Justine , Montréal, QC , Canada.
authors.forename: Elias
authors.initials: E
authors.lastname: Gbeha
authors.affiliation: Sainte-Justine Research Centre, Centre Hospitalier et Universitaire Sainte Justine , Montréal, QC , Canada.
authors.forename: Philip
authors.initials: P
authors.lastname: Awadalla
authors.affiliation: Laboratoire de Biochimie et de Biologie Moléculaire, Faculté des Sciences et Techniques, University of Abomey-Calavi , Cotonou , Benin.
authors.forename: Ambaliou
authors.initials: A
authors.lastname: Sanni
authors.affiliation: Biology Program, Division of Science and Mathematics, New York University Abu Dhabi , Abu Dhabi , United Arab Emirates.
authors.forename: Youssef
authors.initials: Y
authors.lastname: Idaghdour
authors.affiliation: Centre de Prise en charge Médicale Intégrée du Nourrisson et de la Femme Enceinte atteints de Drépanocytose, Faculté des Sciences de la Santé, University of Abomey-Calavi , Cotonou , Benin.
authors.forename: M Cherif
authors.initials: MC
authors.lastname: Rahimy
keywords: anemia; hepcidin; iron deficiency; iron homeostasis; red blood cell indices; serum iron proteins; sickle cell disease