THALASSEMIA IN AZERBAIJAN

beta thalassaemia is present throughout the southern regions of the fo rmer USSR. We have defined the clinical picture of the disorder, the s pectrum of beta thalassaemia mutations, and the role of customary cons anguineous marriage in Azerbaijan, where thalassaemia presents a publi c health problem of the same order as that in Greece. Contrary to earl ier suggestions, we found that the common form of the disorder is typi cally severe. Typical Turkish, Mediterranean, Azeri, Kurdish, and Asia n Indian mutations were found, consistent with the history of the regi on. The common Mediterranean beta0 thalassaemia mutation (codon 39) wa s not found. Three mutations (codon 8-AA, IVS2-1 and IVS1-110) account for over 80% of beta thalassaemia genes. Consanguineous marriage appe ars to contribute relatively little to the frequency of affected birth s. These observations provide the basis for a thalassaemia prevention programme in Azerbaijan.