Iron Overload Disorders: Natural History, Pathogenesis, Diagnosis, and Therapy
| Autor: | Gordon D. McLaren, W. Angus Muir, Robert W. Kellermeyer, Allan Jacobs |
|---|---|
| Rok vydání: | 1983 |
| Předmět: |
Liver Cirrhosis
Pathology medicine.medical_specialty Hemosiderosis Genetic Linkage Anemia Iron Deferoxamine Intestinal absorption Porphyrias Sideroblastic anemia HLA Antigens Liver Cirrhosis Alcoholic Renal Dialysis Humans Medicine Tissue Distribution Porphyria cutanea tarda Mononuclear Phagocyte System Hemochromatosis Bloodletting Chelating Agents Anemia Hypochromic medicine.diagnostic_test business.industry Transferrin Transfusion Reaction Biological Transport General Medicine medicine.disease Pedigree Disease Models Animal Intestinal Absorption Hereditary hemochromatosis Ferritins Serum iron Thalassemia business |
| Zdroj: | CRC Critical Reviews in Clinical Laboratory Sciences. 19:205-266 |
| ISSN: | 0590-8191 |
| DOI: | 10.3109/10408368309165764 |
| Popis: | Hemochromatosis is a syndrome which, when fully expressed, is manifested by melanoderma , diabetes mellitus, and liver cirrhosis, with iron overload involving parenchymal and reticuloendothelial cells in many organ systems. This clinical presentation may arise as a consequence of either hereditary or acquired abnormalities of iron overload, although the mechanisms are quite different. In hereditary hemochromatosis (also known as primary, or idiopathic, hemochromatosis), increased intestinal iron absorption leads to excessive accumulations of iron, throughout the body, particularly in parenchymal cells. In secondary forms of iron overload including transfusional hemosiderosis, alcoholic cirrhosis, thalassemia, sideroblastic anemia, and porphyria cutanea tarda, iron accumulates in the reticuloendothelial system initially, but with increasing amounts of total body iron, excessive iron deposits eventually accumulate in parenchymal cells throughout the body producing a picture indistinguishable from hereditary hemochromatosis. In this article, the course, prognosis, and therapy of iron overload will be reviewed in detail. Clinical and experimental data concerning the pathogenesis of the different forms of iron overload will be examined critically. In particular, information relating to possible abnormalities of reticuloendothelial function, intestinal mucosal iron transport, and alterations in serum and tissue isoferritin patterns in hereditary hemochromatosis will be analyzed, and possible directions for future research will be suggested. The mode of inheritance and linkage with the major histocompatibility (HLA) complex will be discussed. Theories on the pathogenesis of tissue damage by excess iron will be evaluated. Methods for measuring the extent of iron overload in clinical practice will be described, including measurements of serum iron, serum ferritin, iron absorption, cobalt excretion, desferrioxamine excretion, liver biopsy and tissue iron determinations, and HLA typing. Finally, unresolved problems in the understanding of the disease process, diagnosis, and therapy will be delineated. |
| Databáze: | OpenAIRE |
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