Document Detail

Biochemical characterisation of amyloid by endomyocardial biopsy.
MedLine Citation:
PMID:  19291509     Owner:  NLM     Status:  MEDLINE    
Cardiomyopathy is a major cause of death in patients with systemic amyloidosis. There are several forms of systemic amyloidosis which cause cardiomyopathy and determination of the exact type of amyloid in each affected patient is essential for treatment and determination of prognosis. In this study, we tested the feasibility of determining the type of amyloidosis by biochemical analysis of endomyocardial biopsies. Right ventricular endomyocardial biopsies were obtained from 10 patients with restrictive cardiomyopathy. Three patients had monoclonal protein demonstrated in serum or urine and all three had bone marrow findings consistent with monoclonal gammopathy. Seven patients had isolated cardiomyopathy without evidence of monoclonal gammopathy. A portion of each myocardial biopsy was submitted for histologic evaluation and all demonstrated amyloid by Congo red staining. Each biopsy was analysed biochemically by isolation of amyloid fibrils and the protein characterised by amino acid sequence analysis. Four amyloid isolates were characterised as immunoglobulin light chain proteins. Two specimens obtained from patients with transthyretin (TTR) DNA mutations contained TTR peptides proving the hereditary nature of the disease. Biopsies from four patients without a TTR mutation contained TTR and were consistent with the diagnosis of senile cardiac amyloidosis (SCA). All endomyocardial biopsy specimens that were analysed had sufficient amyloid fibril subunit protein to allow characterisation by amino acid sequence analysis. This methodology is particularly useful in differentiating SCA with TTR amyloid fibrils from immunoglobulin light chain amyloidosis which also occurs in the elderly age group.
Merrill D Benson; Jeffrey Breall; Oscar W Cummings; Juris J Liepnieks
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.    
Journal Detail:
Title:  Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis     Volume:  16     ISSN:  1744-2818     ISO Abbreviation:  Amyloid     Publication Date:  2009 Mar 
Date Detail:
Created Date:  2009-03-17     Completed Date:  2009-05-07     Revised Date:  2009-11-19    
Medline Journal Info:
Nlm Unique ID:  9433802     Medline TA:  Amyloid     Country:  United States    
Other Details:
Languages:  eng     Pagination:  9-14     Citation Subset:  IM    
Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN 46202-5126, USA.
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MeSH Terms
Aged, 80 and over
Amino Acid Sequence
Amyloid / chemistry*
Amyloidosis / diagnosis*,  metabolism
Cardiomyopathy, Restrictive / genetics,  metabolism*
Heart Failure / diagnosis
Monoclonal Gammopathy of Undetermined Significance / metabolism
Prealbumin / genetics
Grant Support
Reg. No./Substance:
0/Amyloid; 0/Prealbumin

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