| The natural history of thalassemia intermedia. | |
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MedLine Citation:
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PMID: 20712795 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The severity of thalassemia intermedia depends on the degree of imbalance between alpha and non-alpha chains as well as other genetic and environmental factors that modify the natural history of the disease. By definition, the patients spontaneously maintain hemoglobin at or above 7 g/dL, sometimes at the price of intense hyperplasia of the bone marrow that is in turn responsible for bone deformities, osteoporosis, and extramedullary erythropoietic masses that often characterize thalassemia intermedia. Transfusion may become necessary with advancing age, during infection and pregnancy, and when hypersplenism develops. Splenectomy is often needed. Iron overload in nontransfused patients is due to increased gastrointestinal absorption and involves mainly the liver. Complications affecting the lives of patients with thalassemia intermedia include pulmonary hypertension, leg ulcers, pseudoxanthoma elasticum, gallstones, hepatocellular carcinoma, and thromboembolic events. |
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Authors:
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Caterina Borgna-Pignatti; Maria Marsella; Nicolò Zanforlin |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Annals of the New York Academy of Sciences Volume: 1202 ISSN: 1749-6632 ISO Abbreviation: Ann. N. Y. Acad. Sci. Publication Date: 2010 Aug |
Date Detail:
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Created Date: 2010-08-17 Completed Date: 2010-09-17 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 7506858 Medline TA: Ann N Y Acad Sci Country: United States |
Other Details:
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Languages: eng Pagination: 214-20 Citation Subset: IM |
Affiliation:
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Department of Clinical and Experimental Medicine, Università di Ferrara, Ferrara, Italy. bre@unife.it |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Cardiovascular Diseases
/
etiology,
physiopathology Cholelithiasis / etiology Endocrine System Diseases / etiology, physiopathology Female Hemoglobins / chemistry, metabolism Humans Hypertension, Pulmonary / etiology, physiopathology Iron Overload / etiology, physiopathology Leg Ulcer / etiology Pregnancy Pseudoxanthoma Elasticum / etiology Risk Factors Thalassemia / complications, pathology, physiopathology*, therapy Thrombosis / etiology |
| Chemical | |
Reg. No./Substance:
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0/Hemoglobins |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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