thalassemia…

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thalassemia major, thalassemia minor, thalassemia, mediterranean anemia, cooley's anemia, thalassemias, hereditary disorders characterized by defective production of hemoglobin, leads to low production, excessive destruction, red blood cells, hemoglobin contains two chains, alpha, beta globin, genetic defects inherited, imbalance, in the production of either chain, beta thalassemias, a mutation, in the beta globin chain, genes must be inherited from both parents to acquire the major form of this disease, one gene is inherited, a carrier, not have symptoms, the minor form, major form, children, normal at birth, develop anemia, first year of life, growth failure, bone deformities, enlarged liver, spleen, the problems, blood transfusions, modify some, the disease manifestation, iron overload, transfusions, heart, liver, endocrine systems, mild form of beta thalassemia produces small red blood cells, no symptoms, risk factors, family history of thalassemia, an ethnic background that has shown susceptibility, beta thalassemias occur in people of mediterranean origin, to a lesser extent, chinese, asians, blacks, alpha thalassemias occur most commonly in people from southeast asia, china, deletion of a gene, genes, alpha globin chain, most severe form of alpha thalassemia causes, stillbirth, death of a fetus, delivery, fatigue, shortness of breath, jaundice, bone deformities, in the face, enlarged spleen, peripheral blood smear shows small, abnormally-shaped red blood cells, complete blood count shows anemia, hemoglobin electrophoresis shows abnormal hemoglobin, tests, rbc indices, hemoglobin content, osmotic fragility, severe thalassemia, regular blood transfusions, folate supplementation, people who receive the blood transfusions should avoid iron supplements, oxidative drugs sulfonamides, iron levels, become toxic, patients who receive significant numbers of blood transfusions, require therapy to remove iron, chelation therapy, bone marrow transplant is investigated, a treatment, most successful in children, in severe thalassemia, death from heart failure, occur between the ages of 20, hypertransfusion programs, chelation therapy improve outcome, successful bone marrow transplantation is curative, less severe forms of thalassemia, not shorten life span, untreated, thalassemia major leads to heart failure, liver dysfunction, susceptibility to infection, iron overload, a side effect of treatment, cause damage, heart, liver, endocrine systems, complication is managed by daily injections of an iron chelating agent, binds to iron, to be excreted, in the urine, symptoms develop, suggestive of thalassemia, symptoms develop after treatment, genetic counseling in families, known thalassemia, prenatal screening.