Document Detail

Cardiac amyloidosis: heterogenous pathogenic backgrounds.
MedLine Citation:
PMID:  15645642     Owner:  NLM     Status:  MEDLINE    
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.
Shu-ichi Ikeda
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Internal medicine (Tokyo, Japan)     Volume:  43     ISSN:  0918-2918     ISO Abbreviation:  Intern. Med.     Publication Date:  2004 Dec 
Date Detail:
Created Date:  2005-01-13     Completed Date:  2005-03-16     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  9204241     Medline TA:  Intern Med     Country:  Japan    
Other Details:
Languages:  eng     Pagination:  1107-14     Citation Subset:  IM    
Third Department of Medicine, Shinshu University School of Medicine, Matsumoto.
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MeSH Terms
Amyloidosis* / classification,  diagnosis,  therapy
Amyloidosis, Familial / diagnosis,  therapy
Cardiomyopathies* / diagnosis,  therapy

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