beta-thalassemia


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Related to beta-thalassemia: Alpha-thalassemia, Thalassemia minor

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.

beta-thalassemia

an anemia that is caused by diminished synthesis of beta chains of hemoglobin. The homozygous form is known as thalassemia major and the heterozygous form is known as thalassemia minor. See 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

beta-thalassemia (bāˑ·t-tha·ls·sēˑ·mē·),

n type of anemia occurring as a result of reduced synthesis of the beta chains of hemoglobin.

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 ?
Different hematological phenotypes caused by the interaction of triplicated alpha-globin genes and heterozygous beta-thalassemia.
Molecular study and prenatal diagnosis of alpha- and beta-thalassemias in Chinese.
Molecular diagnosis of beta-thalassemia by multiplex ARMS-PCR: a cost effective method for developing countries like Pakistan.
Experts have known for some time that inhibiting or reversing gamma-to-beta switching of hemoglobin subunits can raise levels of fetal hemoglobin in adults and significantly ameliorate the debilitating symptoms of beta-thalassemia or sickle cell disease.
Patients with beta-thalassemia major require repeated red blood cells transfusion therapy in order to maintain their haemoglobin level between 10-12 gm/dL and survive.
In beta-thalassemia, a long-studied genetic disorder, a mutation impairs hemoglobin production, resulting in defective red blood cells (RBCs).
Metabolic and endocrinologic complications in beta-thalassemia major: a multicenter study in Tehran," BMC Endocrine Disorders, vol.
Audiological evaluation in adult beta-thalassemia major patients under regular chelation treatment.
Understanding Beta-Thalassemia with Focus on the Indian Subcontinent and the Middle East.
Incidence rate of major beta-thalassemia and study of its causes after prevention and control program of thalassemia in Isfahan province.
However, we observed no significant association between the survival time of beta-thalassemia and consanguinity.