Abstract
Control studies about patients suspected for thalassemia major revealed 8 cases homozygous for beta-thalassemia in 6 families: 3 of them produce no beta A globin at all (beta thalassemia type) and 5 others synthesize various amounts of beta chain (beta+ thalassemia type).Non alpha/alpha ratios were calculated after incubations of reticulocytes in vitro.They were very similar in the two types,changing from 0,13 to 0,34. However levels of HbF and alpha globin chain synthesis greatly differ in the beta+ thalassemia type (HbF percentage and alpha, non alpha ratios changing from 9 % to 67 % and 0,16 to 0,73 respectively.
7 cases from 4 families (association of beta-thalassemia and HbS) have been also studied: 4 cases produce only beta S globin chains (betao thalassemia/beta S) and 3 others synthesize beta S and some beta A globin chains (beta+ thalassemia/ beta S).
Here also non alpha/alpha ratios did not differ between the two types and vary from 0,37 to 0,43.However in the beta+ thalassemia/beta S cases one member of family more affected by anemia needed more transfusions.
Further clinical and biochemical characterization are now in progress in order to attempt some better classification of beta+ thalassemia in West-Algeria.
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Moralé, L., Morlé, F., Bouhass, R. et al. Some aspects of beta-thalassemia in Wes6-Algeria: 96. Pediatr Res 14, 180 (1980). https://doi.org/10.1203/00006450-198002000-00123
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DOI: https://doi.org/10.1203/00006450-198002000-00123