Alpha-thalassemia


Also found in: Dictionary, Thesaurus, Encyclopedia.
Related to Alpha-thalassemia: Beta-thalassemia, hemoglobin H disease

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.

α-thalassemia

Hemoglobin Barts Hematology An inherited condition caused by a defect in the synthesis of the Hb α chain; Hb Barts hemoglobinopathy is characterized by the presence of 4 gamma chains; it is more common in southeast Asians; the most severe form of alpha thalassemia causes stillbirth due to hydrops fetalis Heredity Parents are carriers–heterozygotes; one in 4 children is homozygous for the mutation and thus has full-blown disease Clinical Pallor, fatiguability, FTT, fever, infections, diarrhea Management Transfusions

Alpha-thalassemia

An inherited disorder that interferes with the normal production of hemoglobin.
References in periodicals archive ?
Brown, "Cerebro-vascular accidents in children with sickle-cell disease and alpha-thalassemia," The Journal of Pediatrics, vol.
Genotyping of alpha-thalassemia in microcytic hypochromic anemia patients from North India.
In the mid-western region, the migratory target home of Brazilians from several regions of the country, a sample of 404 individuals from 55 cities in the state of Goias revealed a 10.1% prevalence of hereditary anemia, thalassemia and hemoglobin variants, specifically 5.2% of heterozygous alpha thalassemia, 2.2% of hemoglobin S heterozygosity (HbAS); 1% heterozygosity for hemoglobin C (HbAC); 0.7% of lower beta thalassemia; 0.5% alpha-thalassemia and heterozygous for HbS; 0.3% alpha-thalassemia and heterozygous for HbC; and 0.3% heterozygous for hemoglobin D (HbAD) (MELO-REIS et al., 2006).
Diversity of alpha-globin mutations and clinical presentation of alpha-thalassemia in Israel.
In this study, we used the automatic HPLC system set up with the alpha-thalassemia short (ATS) program that we developed and with the beta-thalassemia short (BTS) program to detect various types of thalassemias in both prenatal and postnatal specimens.
I read the article on the "The incidence of alpha-thalassemia in Iraqi Turks".
Alpha thalassemia is insidious in that the hemoglobin phenotype in an alpha-thalassemia trait patient is completely normal.
More insidious social costs of iron deficiency are limitation in intellectual development in children6 and impaired work performance in adults, which together affects social and economic development.7 ID usually develops in a sequential manner over a period of negative iron balance, such as periods of blood loss and/or prolonged iron-deficient diet, accelerated growth in children and adolescents as well as during pregnancy and lactation.8,9 Thalassemia trait is also frequently misdiagnosed as iron deficiency in children because the two are similar hematologically and iron deficiency is much more prevalent.10 Alpha-Thalassemia trait is characterized by marked microcytosis and hypochromia of red cells in conjunction with mild anaemia and erythrocytosis.
Alpha-thalassemia affects the synthesis of alpha globin chains, and the severity of disease is dependent on the extent of gene deletion.