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1. Glucose 6-phosphate dehydrogenase deficiency and sickle cell anemia: frequency and features of the association in an African community

Author

Bienzle, U, Sodeinde, O, Effiong, CE, and Luzzatto, L

Abstract

The glucose 6-phosphate dehydrogenase (G6PD) genotype was determined in 100 male patients with homozygous sickle cell anemia (SS) by a combination of quantitative assay, cytochemical testing, and starch-gel electrophoresis. Of the 100 patients tested, 16 were found to be G6PD deficient (GdA-), AND 84 G6PD normal (22GsA and 62 GdB). This distribution of G6PD genotypes did not differ significantly from that observed in the general population. The level of G6PD activity in GdA- SS patients was nearly always higher than in G6PD-deficient subjects who did not have an associated hemolytic state, but it was nearly always lower than in G6PD-normal subjects. The clinical course of sickle cell disease, including the degree of anemia, was not milder in GdA- than in G6PD-normal patients but could not be proved to be significantly more severe. It was concluded that in this community the incidence of G6PD deficiency in sickle cell anemia was not greater than would be expected by chance, and there was no evidence that the coexistence of the GdA- gene in SS patients ameliorated their disease.

Published
1975
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2. Circulatory hepcidin is associated with the anti-inflammatory response but not with iron or anemic status in childhood malaria.

Author

Burté F, Brown BJ, Orimadegun AE, Ajetunmobi WA, Afolabi NK, Akinkunmi F, Kowobari O, Omokhodion S, Osinusi K, Akinbami FO, Shokunbi WA, Sodeinde O, and Fernandez-Reyes D

Subjects
Anemia blood, Anemia complications, Case-Control Studies, Child, Child, Preschool, Female, Ferritins blood, Hematocrit, Hepcidins, Humans, Interleukin-10 blood, Interleukin-6 blood, Iron blood, Linear Models, Malaria, Cerebral complications, Malaria, Falciparum complications, Male, Nigeria, Prospective Studies, Receptors, Transferrin blood, Tertiary Care Centers, Transferrin analysis, Antimicrobial Cationic Peptides blood, Cytokines blood, Inflammation Mediators blood, Malaria, Cerebral blood, Malaria, Falciparum blood
Abstract

Cerebral malaria (CM) and severe malarial anemia (SMA) are the most serious life-threatening clinical syndromes of Plasmodium falciparum infection in childhood. Therefore, it is important to understand the pathology underlying the development of CM and SMA as opposed to uncomplicated malaria (UM). Increased levels of hepcidin have been associated with UM, but its level and role in severe malarial disease remains to be investigated. Plasma and clinical data were obtained as part of a prospective case-control study of severe childhood malaria at the main tertiary hospital of the city of Ibadan, Nigeria. Here, we report that hepcidin levels are lower in children with SMA or CM than in those with milder outcome (UM). While different profiles of pro- and anti-inflammatory cytokines were observed between the malaria syndromes, circulatory hepcidin levels remained associated with the levels of its regulatory cytokine interleukin-6 and of the anti-inflammatory cytokine inerleukin-10, irrespective of iron status, anemic status, and general acute-phase response. We propose a role for hepcidin in anti-inflammatory processes in childhood malaria.

Published
2013
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