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.
Subject(s)
Emigrants and Immigrants , Glucosephosphate Dehydrogenase Deficiency/epidemiology , Hemoglobinopathies/epidemiology , Adolescent , Adult , Africa South of the Sahara/ethnology , Aged , Child , Child, Preschool , Female , Humans , Infant , Male , Middle Aged , Prevalence , Spain/epidemiologyABSTRACT
FUNDAMENTO Y OBJETIVO: Es conocido que las prevalencias de hemoglobinopatía S y de déficit de glucosa-6-fosfatodeshidrogenasa (G6PD) son muy elevadas en determinadas zonas del África occidental debido a la presiónpositiva que ejerce la malaria en estos defectos congénitos. La presencia en el Maresme, una comarca de laCataluña oriental, de un gran número de inmigrantes africanos procedentes de estas zonas puede permitir conocer la prevalencia real de estas 2 alteraciones eritrocitarias en dicha población.POBLACIÓN Y MÉTODO: Se ha estudiado a 204 personas de raza negra que acudieron de forma consecutiva a laUnidad de Atención al Inmigrante o a las Consultas Externas del Consorcio Sanitario de Mataró por un motivode consulta distinto de anemia. Se realizaron a todos ellos hemograma, electroforesis de hemoglobina en acetatode celulosa, 2 pruebas de escrutinio para el déficit de G6PD y la determinación específica de esta enzimamediante espectrofotometría.RESULTADOS: Se estudió a 141 varones (69%) y a 63 mujeres (31%), con una media de edad de 30,8 años(rango: 1-70). Procedían de Gambia, Senegal, Guinea Ecuatorial, Guinea-Bissau, Malí, Somalia, Guinea-Conakry,Nigeria, Ghana, Costa de Marfil, Sierra Leona, Liberia y Mauritania, y pertenecían a las etnias sarankholé,mandinga, fula, peul, diola, bambara, pare e ibo (por orden de frecuencia). Se detectaron alteracionesde la hemoglobina en 44 individuos (21,5%): 36 portadores de hemoglobina S heterocigotos (17,6%) y 8 dehemoglobina C 7 heterocigotos (3,9%) y uno homocigoto (0,49%). No se encontró ninguna otra hemoglobinopatía.Asimismo, se identificó a 31 personas con déficit de G6PD. Según los resultados obtenidos, la prevalenciade hemoglobinopatía S en la población estudiada es del 17,6%; la de hemoglobinopatía C, del 3,9%, yla de déficit de G6PD, del 15,2%. La asociación de déficit de G6PD y hemoglobinopatías únicamente se hallóen un 3% de los casos (en un 2,5% con hemoglobinopatía S y en un 0,5% con hemoglobinopatía C).CONCLUSIONES: Los resultados demuestran una alta prevalencia de hemoglobinopatías y déficit de G6PD en esta población. La morbilidad y mortalidad de la drepanocitosis, y las complicaciones debidas al déficit de G6PD, junto con la fácil detección mediante electroforesis de hemoglobina y determinación de G6PD, hacen aconsejable realizar estas exploraciones de forma sistemática en las zonas que atiendan a población subsahariana
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 Matarófor any medical or surgical problems but not for anemia. The G6PD deficiency was determined by a quantitativeassay 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). Thestudied 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 foundabnormal 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 is3.9%, and the prevalence of G6PD deficiency is 15.2%. The association of abnormal hemoglobins and G6PDdeficiency 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 thispopulation. 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 standardizethese tests in areas with high density of black people
Subject(s)
Humans , Hemoglobinopathies/epidemiology , Glycogen Storage Disease Type I/epidemiology , Hemoglobinopathies/physiopathology , Glycogen Storage Disease Type I/physiopathology , Emigration and Immigration , Sickle Cell Trait/epidemiology , Africa South of the Sahara/epidemiology , Sex Distribution , Age DistributionSubject(s)
Anemia, Pernicious/diagnosis , Folic Acid/blood , Vitamin B 12 Deficiency/diagnosis , Anemia, Macrocytic/blood , Anemia, Macrocytic/drug therapy , Anemia, Pernicious/blood , Anxiety/etiology , False Negative Reactions , Folic Acid/pharmacology , Folic Acid/therapeutic use , Gait Disorders, Neurologic/etiology , Humans , Male , Middle Aged , Paresthesia/etiology , Vitamin B 12 Deficiency/blood , Vitamin B 12 Deficiency/complicationsABSTRACT
No disponible
Subject(s)
Male , Middle Aged , Humans , Anemia, Pernicious/diagnosis , Vitamin B 12 Deficiency/diagnosis , Folic Acid/therapeutic use , Anemia, Pernicious/drug therapyABSTRACT
No disponible
