title: Biological and clinical presentation of patients with hemoglobinopathies attending an urban hospital in Ouagadougou: confirmation of the modification of the balance between Hb S and Hb C in Burkina Faso.
abstract: The incidence of hemoglobinopathies (Hb C and Hb S) is relatively high in West Africa. In order to characterize the clinical phenotypes of these hemoglobinopathies 10,166 subjects were studied for suspected hemoglobinopathies at the Laboratory of the Centre Medical Saint Camille (CMSC), Ouagadougou, Burkina Faso. A high rate of Hb SC (6.49%) and Hb SS (1.93%) individuals were detected at the CMSC as a consequence of a selective process, whereby patients with anemia or symptoms of vascular occlusive crisis underwent blood tests. The higher frequency of Hb SC may be explained by the fact that this condition is less severe than the SS status, and it requires frequent clinical and laboratory review. On the other hand, the frequency of Hb CC is very low because it does not interfere with their health status. Moreover, the high percentage of Hb S (12.29%) and Hb C (19.28%) trait individuals may be explained by the fact that, in general, all Hb SS and Hb SC patients followed at the CMSC have parents, siblings and other relatives who could have been referred by the center to receive blood tests. The dramatic increase over the past few years in the prevalence of Hb SS [who were absent in the 1984 study of Labie et al. [5]] and of Hb SC, may be attributed to its reduced lethality due to social and health changes. In conclusion, secondary prevention for the control of concurrent and associated diseases is essential in Hb SS and Hb SC patients for improving health and life expectancy.
authors.affiliation: Centre Medical Saint Camille, Ouagadougou, Burkina Faso.
authors.forename: Jacques
authors.initials: J
authors.lastname: Simpore
authors.forename: Salvatore
authors.initials: S
authors.lastname: Pignatelli
authors.forename: Sergio
authors.initials: S
authors.lastname: Barlati
authors.forename: Salvatore
authors.initials: S
authors.lastname: Musumeci
mesh_terms: D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics; D002050:Burkina Faso / Q000453:epidemiology; D002648:Child; D003695:Delivery of Health Care; D005787:Gene Frequency; D005820:Genetic Testing; D005838:Genotype; D006445:Hemoglobin C Disease / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics; D006453:Hemoglobinopathies / Q000175:diagnosis / Q000453:epidemiology* / Q000235:genetics; D006786:Hospitals, Urban; D006801:Humans; D015995:Prevalence; D012423:Rural Health; D014504:Urban Health