| Cardiac amyloidosis: heterogenous pathogenic backgrounds. | |
MedLine Citation:
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PMID: 15645642 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Cardiac amyloidosis is a fatal disorder which develops on the basis of the different pathologic conditions in systemic amyloidosis: the most common underlying disease is immunoglobulin light chain-derived primary amyloidosis and the next is transthyretin-related hereditary amyloidosis; the latter disorder, typically represented by familial amyloid polyneuropathy, was long regarded as an endemic disease. However, this disorder has now been shown to involve a highly variable clinical picture due to a large number of transthyretin gene mutations, and many patients with diverse ancestors suffer from severe cardiac amyloidosis. Additionally, senile systemic amyloidosis is now noted as a cause of cardiac dysfunction in elderly individuals. Echocardiogram and myocardial technetium-99m-pyrophosphate scintigraphy can provide characteristic findings. Immunohistochemistry on tissue amyloid, biochemical analysis of serum and urine proteins, and DNA sequencing are usually employed to determine the disease-related amyloid fibril protein. Although systemic amyloidosis has become treatable, the prognosis of each patient who received up-to-date and effective, but nevertheless stressful, therapy depends on the severity of cardiac involvement by amyloid deposition. |
Authors:
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Shu-ichi Ikeda |
Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't; Review |
Journal Detail:
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Title: Internal medicine (Tokyo, Japan) Volume: 43 ISSN: 0918-2918 ISO Abbreviation: Intern. Med. Publication Date: 2004 Dec |
Date Detail:
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Created Date: 2005-01-13 Completed Date: 2005-03-16 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 9204241 Medline TA: Intern Med Country: Japan |
Other Details:
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Languages: eng Pagination: 1107-14 Citation Subset: IM |
Affiliation:
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Third Department of Medicine, Shinshu University School of Medicine, Matsumoto. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Amyloidosis*
/
classification,
diagnosis,
therapy Amyloidosis, Familial / diagnosis, therapy Cardiomyopathies* / diagnosis, therapy Humans |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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