Beta-thalassemia in Yugoslavia

Hemoglobin. 1990;14(1):15-24. doi: 10.3109/03630269009002251.

Abstract

This study concerned the evaluation of beta-thalassemia alleles in nearly 50 patients with beta-thalassemia major and in 130 -thalassemia heterozygotes using gene amplification and dot-blot hybridization with synthetic probes. Fourteen different mutations were observed; of these, three (IVS-I-110; IVS-I-6; IVS-I-1) account for some 75% of all beta-thalassemia alleles. Newly discovered variants, i.e. T----C in the initiation codon and AATAAA----AATGAA in the poly A site were observed in a few patients. The poly A mutation with classical beta-thalassemia alleles result in thalassemia intermedia. Hb Lepore is a rather common abnormality and combinations of this variant with beta-thalassemia often result in severe disease; a search for beta-thalassemia mutations among patients affected with this disease should include an analysis to detect this hemoglobin abnormality.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Alleles
  • Base Sequence
  • DNA Mutational Analysis
  • Gene Frequency
  • Genes
  • Genotype
  • Globins / genetics*
  • Hemoglobinopathies / complications
  • Hemoglobins, Abnormal / genetics*
  • Humans
  • Thalassemia / complications
  • Thalassemia / epidemiology*
  • Thalassemia / genetics
  • Yugoslavia / epidemiology

Substances

  • Hemoglobins, Abnormal
  • Globins