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Paperback Beta-Thalassemia Book

ISBN: 551221246X

ISBN13: 9785512212462

Beta-Thalassemia

High Quality Content by WIKIPEDIA articles Beta-thalassemias (β-thalassemias) are a group of inherited blood disorders caused by reduced or absent synthesis of the beta chains of hemoglobin resulting in variable phenotypes ranging from severe anemia to clinically asymptomatic individuals. The total annual incidence of symptomatic individuals is estimated at 1 in 100,000 throughout the world. Three main forms have been described: thalassemia major, thalassemia intermedia and thalassemia minor. Individuals with beta thalassemia major usually present within the first two years of life with severe anemia, poor growth, and skeletal abnormalities during infancy. Affected children will require regular lifelong blood transfusions. Beta thalassemia intermedia is less severe than beta thalassemia major and may require episodic blood transfusions. Transfusion-dependent patients will develop iron overload and require chelation therapy to remove the excess iron. Bone marrow transplants can be curative for some children with beta thalassemia major. Transmission is autosomal recessive; however, dominant mutations have also been reported. Genetic counseling is recommended and prenatal diagnosis may be offered.

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