Homozygous beta thalassaemia in Liberia.
Open Access
- 1 June 1975
- journal article
- Published by BMJ in Journal of Medical Genetics
- Vol. 12 (2), 165-173
- https://doi.org/10.1136/jmg.12.2.165
Abstract
The clinical and haematological findings in 19 Liberians probably homozygous for beta thalassaemia are described. The haemoglobin patterns were similar with Hb F levels in the 30-50% range and a raised level of Hb A2 and, although the clinical severity varied widely, over half the cases were symptomless and even the more severely affected ones showed a milder picture than that found in Mediterranean races. Haemoglobin-synthesis studies carried out on three homozygotes and two heterozygotes indicated a variable degree of globin-chain imbalance. The reasons for the mild course of the disease in Liberians and other African races are discussed; it is likely that the beta-thalassaemia genes in these populations are different from those in other racial groups. It is noted that all persons in this study belong to tribes which have a low incidence of the sickle-cell gene.Keywords
This publication has 16 references indexed in Scilit:
- Thalassaemia in northern Liberia. A survey in the Mount Nimba area.Journal of Medical Genetics, 1975
- Thalassaemia in the BritishBMJ, 1973
- Thalassaemia Major with Complete Suppression of HbA Production in a Ghanaian GirlActa Haematologica, 1972
- Probable Homozygous Beta Thalassemia in a Negro ChildArchives of Pediatrics & Adolescent Medicine, 1970
- The Thalassaemia Syndromes in NigeriaBritish Journal of Haematology, 1970
- Mild thalassemia: the result of interactions of alpha and beta thalassemia genesJCI Insight, 1970
- The Pattern of Disordered Haemoglobin Synthesis in Homozygous and Heterozygous β-ThalassaemiaBritish Journal of Haematology, 1969
- Globin Synthesis in Thalassaemia: An in vitro StudyNature, 1965
- Biochemical Phenotypes of Thalassemia in the American Negro Population*Annals of the New York Academy of Sciences, 1964
- Thalassaemia in LiberiaBMJ, 1959