beta-thalassemia

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thalassemia

 [thal″ah-se´me-ah]
a heterogeneous group of hereditary hemolytic anemias marked by a decreased rate of synthesis of one or more hemoglobin polypeptide chains, classified according to the chain involved (α, β, δ); the two major categories are α- and β-thalassemia.
α-thalassemia (alpha-thalassemia) that caused by diminished synthesis of alpha chains of hemoglobin. The homozygous form is incompatible with life, the stillborn infant displaying severe hydrops fetalis. The heterozygous form may be asymptomatic or marked by mild anemia.
β-thalassemia (beta-thalassemia) that caused by diminished synthesis of beta chains of hemoglobin. The homozygous form is called t. major and the heterozygous form is called t. minor.
thalassemia ma´jor the homozygous form of β-thalassemia, in which hemoglobin A is completely absent; it appears in the newborn period and is marked by hemolytic, hypochromic, microcytic anemia; hepatosplenomegaly; skeletal deformation; mongoloid facies; and cardiac enlargement.
thalassemia mi´nor the heterozygous form of β-thalassemia; it is usually asymptomatic, but there may be mild anemia.
sickle cell–thalassemia a hereditary anemia involving simultaneous heterozygosity for hemoglobin S and thalassemia.

β-thalassemia

Thalassemia major Hematology A hemoglobinopathy caused by a defect in the synthesis of Hb β chain Clinical Pallor, fatigability, FTT, fever due to infections, diarrhea, bone deformities, hepatosplenomegaly Management Transfusions, but iron overload can damage the heart, liver, and endocrine systems, ergo iron chelation–early use of deferiprone, deferoxamine ↓ transfusion-related iron overload and may protect against DM, cardiac disease, early death

be·ta-thal·as·se·mi·a

(bātă thală-sēmē-ă)
Thalassemia due to one of two or more genes that depress (partially or completely) synthesis of beta-globin chains by the chromosome bearing the abnormal gene.
References in periodicals archive ?
Cell and Gene Therapy for the Beta-Thalassemias: Advances and Prospects.
Beta-Thalassemia: A world of complications with new alternatives of treatment
Nadkarni, "Global burden, distribution and prevention of beta-thalassemias and hemoglobin E disorders," Expert Review of Hematology, vol.
Canatan et al., "beta-Thalassemia Distribution in the Old World: an Ancient Disease Seen from a Historical Standpoint," Mediterranean Journal of Hematology and Infectious Diseases, vol.
Molecular study and prenatal diagnosis of alpha- and beta-thalassemias in Chinese.
A 12-year preventive program for beta-thalassemia in Northern Sardinia.
Beta-thalassemias are a group of hereditary blood disorders characterized by anomalies in the synthesis of the beta chains of hemoglobin resulting in variable phenotypes ranging from severe anemia to clinically asymptomatic individuals.
Beta-Thalassemias are the most important among the Thalassemia syndromes and have become a world wide clinical problem due to an increasing immigrant population.
Methodology: This study was performed on 244 patients with major beta-Thalassemia admitted in Taleghani hospital of Gorgan between 2000 and 2007.
Reactivation of fetal hemoglobin in patients with beta-thalassemia. Semin Hematol.
Beta-thalassemia is the classical form of thalassemias and was first described by Dr.