[Hemoglobinopathies and glucose-6-phosphate dehydrogenase deficiency in the sub-Saharan immigrant population of the Center and South Maresme region, Catalonia, Spain]

Abstract

Background and objective: Prevalence of hemoglobinophaty S and glucose-6-phosphate dehydrogenase (G6PD) deficiency are very increased in certain zones of Western Africa due to the positive pressure exerted by malaria on these congenital defects. In Maresme, a region of East Catalonia, there is a numerous community of African people, coming from areas of Western Africa where sickle cell trait and G6PD deficiency are frequent. The aim of our study was to know the prevalence of both disturbances in this population.

Population and method: We studied 204 individuals of black ethnical background who were migrant Africans. They attended the Immigrant Attention Unit or Outside Surgery Department of Consorcio Sanitario de Mataró for any medical or surgical problems but not for anemia. The G6PD deficiency was determined by a quantitative assay and 2 screening techniques. The identification of abnormal hemoglobins was done by electrophoresis.

Results: We studied 141 males (69%) and 63 females (31%). Mean age was 30.8 years (range: 1-70). The studied population came from Gambia, Senegal, Equatorial Guinea, Guinea Bissau, Mali, Somalia, Guinea Conakry, Nigeria, Ghana, Ivory Coast, Sierra Leone, Liberia and Mauritania, and were members of the Sarankhole, Mande, Fulani, Peul, Djola, Bambara, Pare and Ibo ethnic groups (according to the frequency order). We found abnormal hemoglobins in 44 subjects (21.5%): 36 hemoglobin S carriers, 7 hemoglobin C carriers and one hemoglobin C homozygous. Moreover, we identified G6PD deficiency in 31 subjects. According to these results, the prevalence of hemoglobinophaty S in these people is 17.6%; the prevalence of hemoglobinophaty C is 3.9%, and the prevalence of G6PD deficiency is 15.2%. The association of abnormal hemoglobins and G6PD deficiency was found only in a 3% of the cases (2.5% hemoglobinophaty S and 0.5% hemoglobinophaty C).

Conclusions: This results demonstrate a high prevalence of hemoglobinopathies and G6PD deficiency in this population. The morbidity and mortality of sickle cell disease and the complications due to G6PD deficiency, besides the easy detection using electrophoresis and G6PD determination, make it necessary to standardize these tests in areas with high density of black people.