Genetic counseling for sickle cell anemia and hemoglobin C disease in populations where preferential marriage occurs (research project)
Chaventre, A.; Fofana, Y.
Journal de Genetique Humaine 28(2): 91-95
1980
ISSN/ISBN: 0021-7743 PMID: 7463013 Document Number: 157587
Hemoglobinosis S or drepanocytosis is a disorder that generally occurs after the age of 6 mo., with hemolytic anemia, evolving with pains of abdominal and dorsal sites and of the extremities. Without therapy, the prognosis is serious, often fatal before age 4. The disease is hereditary, with an autosomal recessive mode of inheritance. Individuals affected are homozygotes for a gene responsible for the synthesis of 80-100% abnormal Hb. Hemoglobinosis C has the same mode of tramsmission as the S form, but the hemolytic anemia is much less severe. The 2 Hb are most often present in the same populations and give rise to double hemoglobinopathies SC and SS.