Deferoksamin Untuk Pencegahan hemokromatosis pada penderita thalassemia

Thalassemia major is an inherited diiorder of globin synthesis characterized by in effective erythropoiesis, shortened red blood cell survival, and severe anemia. Repeated transfusions are the basis of therapy for this disease. Although such therapy alleviates the anemia and its sequelae, it leads t...

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Main Author: Perpustakaan UGM, i-lib
Format: Article
Published: [Yogyakarta] : Universitas Gadjah Mada 1993
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author Perpustakaan UGM, i-lib
author_facet Perpustakaan UGM, i-lib
author_sort Perpustakaan UGM, i-lib
collection UGM
description Thalassemia major is an inherited diiorder of globin synthesis characterized by in effective erythropoiesis, shortened red blood cell survival, and severe anemia. Repeated transfusions are the basis of therapy for this disease. Although such therapy alleviates the anemia and its sequelae, it leads to massive iron deposition to the body tissue or hemochromatosis. These patients undergo progressive deterioration in pancreatic, hepatic and cardiac. function and usually succumb to life-theaterning arrhythmias or intractable heart failure. In the last decade, children with thalassemia major have been treated with an intensive transfusion regimen combined with continuous iron chelation with deferoxamine. Excretion of iron in response to intramuscular deferoxamine, however, is less than that achieved in response to continuous subcutaneous of intravenous infusions. Subcutaneous administration of deferoxamine is nearly (79%90%) as effective as the intravenous route, and appears to be the methode of choice. The recomended dose of deferoxamine was 20.60 mg/kg body weight. Vitamin C supplementation augments excretion of iron in response to deferoxamine in many patients. Treatment with deferoxamine has prove delay cardiac complication, prevent hepatic fibrosis, improve growth hormon secretion and survival time for patients with thalassemia major. Key words: thalassemia, hemochromatosis, deferoxamine, Vit. C, survival time
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spelling oai:generic.eprints.org:186152014-06-18T00:39:54Z https://repository.ugm.ac.id/18615/ Deferoksamin Untuk Pencegahan hemokromatosis pada penderita thalassemia Perpustakaan UGM, i-lib Jurnal i-lib UGM Thalassemia major is an inherited diiorder of globin synthesis characterized by in effective erythropoiesis, shortened red blood cell survival, and severe anemia. Repeated transfusions are the basis of therapy for this disease. Although such therapy alleviates the anemia and its sequelae, it leads to massive iron deposition to the body tissue or hemochromatosis. These patients undergo progressive deterioration in pancreatic, hepatic and cardiac. function and usually succumb to life-theaterning arrhythmias or intractable heart failure. In the last decade, children with thalassemia major have been treated with an intensive transfusion regimen combined with continuous iron chelation with deferoxamine. Excretion of iron in response to intramuscular deferoxamine, however, is less than that achieved in response to continuous subcutaneous of intravenous infusions. Subcutaneous administration of deferoxamine is nearly (79%90%) as effective as the intravenous route, and appears to be the methode of choice. The recomended dose of deferoxamine was 20.60 mg/kg body weight. Vitamin C supplementation augments excretion of iron in response to deferoxamine in many patients. Treatment with deferoxamine has prove delay cardiac complication, prevent hepatic fibrosis, improve growth hormon secretion and survival time for patients with thalassemia major. Key words: thalassemia, hemochromatosis, deferoxamine, Vit. C, survival time [Yogyakarta] : Universitas Gadjah Mada 1993 Article NonPeerReviewed Perpustakaan UGM, i-lib (1993) Deferoksamin Untuk Pencegahan hemokromatosis pada penderita thalassemia. Jurnal i-lib UGM. http://i-lib.ugm.ac.id/jurnal/download.php?dataId=1410
spellingShingle Jurnal i-lib UGM
Perpustakaan UGM, i-lib
Deferoksamin Untuk Pencegahan hemokromatosis pada penderita thalassemia
title Deferoksamin Untuk Pencegahan hemokromatosis pada penderita thalassemia
title_full Deferoksamin Untuk Pencegahan hemokromatosis pada penderita thalassemia
title_fullStr Deferoksamin Untuk Pencegahan hemokromatosis pada penderita thalassemia
title_full_unstemmed Deferoksamin Untuk Pencegahan hemokromatosis pada penderita thalassemia
title_short Deferoksamin Untuk Pencegahan hemokromatosis pada penderita thalassemia
title_sort deferoksamin untuk pencegahan hemokromatosis pada penderita thalassemia
topic Jurnal i-lib UGM
work_keys_str_mv AT perpustakaanugmilib deferoksaminuntukpencegahanhemokromatosispadapenderitathalassemia