Alpha Thalassemia Disorders
نویسندگان
چکیده
The thalassaemias, the commonest monogenic diseases, are a family of inherited disorders of haemoglobin synthesis characterised by a reduced output of one or other of the globin chains of adult haemoglobin. They are likely to pose an increasing health problem for many developing countries during the early part of the new millennium (1). This review focuses mainly on their control and management, a subject of increasing importance not only for parts of the world in which the disease is particularly common but for any country which has an immigrant population from these regions.
منابع مشابه
بررسی پراکندگی موتاسیون های آلفا تالاسمی در مزدوجین مشکوک نهایی شهرستان بابلسر 1385 -91
Background and purpose: Alpha thalassemia is one of the most common hemoglobin disorders. Some combination of alpha globin gene mutations may cause HbH disease with severe anemia or intermediate thalassemia. In this study we aimed to determine the spectrum of alpha globin gene mutations especially rare mutation at alpha carrier couples in Babolsar, north of Iran. Discovering this spectrum i...
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Background: The aim of this perspective study was to assess the frequency of hemoglobinopathy mutational genes among voluntary hemoglobinopathy carrier couples-to-be referred to thalassemia center, Shafa hospital, affiliated to Ahvaz Jondidishapur University of Medical Sciences (AJUOMS), during their first trimester of pregnancy for genetic screening and counseling for prenatal diagnosis (PND)....
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Alpha Thalassemia is one of the most prevalent disorders worldwide with a [T1] high carrier rate in Mazandaran province (north of Iran). Carriers of --MED double gene deletion are at risk of having a child with hemoglobin haemoglobin[T2] H (HbH) disease, if they marry a silent carrier. Co-inheritance of αααAnti3.7 triplication that cannot be detected using hem...
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Introduction: Newborn screening is a systematic application of tests for early detection, diagnosis, and treatment of certain genetic or metabolic disorders that may lead to mortality and morbidity if untreated. As stated by WHO, each year over 330,000 babies are born worldwide with a severe form of hemoglobinopathy. Newborn screening for hemoglobinopathies could become one of the most importan...
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Background: In various cancers, Ganoderic Acid A (GAA), an active triterpenoid derived from Ganoderma Background: Thalassemia refers to a category of inherited disorders resulting from defects in synthesizing one or several chains of hemoglobin (Hb). The present study aimed to determine the frequency of alpha and beta-thalassemia mutations in Kurdistan province, Iran. Materials and Methods: In...
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Thalassemias are a group of inherited disorders that are characterized by decreased production of the alpha or beta globin chains. The amount of the produced normal hemoglobin within the red blood cells correlates with the severity of the symptoms. Fetuses with alpha-thalassemia major usually die. Children with beta-thalassemia major are usually dependent on repeated blood transfusions. Splenec...
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