Document Detail

Nephrogenic systemic fibrosis.
MedLine Citation:
PMID:  18663283     Owner:  NLM     Status:  MEDLINE    
Nephrogenic systemic fibrosis is a recently diagnosed disease that occurs in patients with chronic kidney disease and acute renal failure. The patients develop skin thickening and fibrosis which is usually symmetrical, and typically of the upper and lower extremities. In some cases the progression is rapid leading to joint contractures confining the patient to a wheelchair. Systemic involvement may occur, leading to cardiomyopathy, pulmonary fibrosis, pulmonary hypertension, diaphragmatic paralysis and in severe cases death. The pathophysiology of the disease still remains unclear, but recent studies have demonstrated gadolinium deposits in tissues of patients diagnosed with nephrogenic systemic fibrosis. The prevalence of nephrogenic systemic fibrosis after exposure to gadolinium has been reported to be up to 12% in CKD stage 5 patients after a single exposure. No single treatment has been shown to be effective, although there are some patients shown to have improvement of their clinical symptoms with regaining of renal function especially after transplantation. In this article we review the current literature of this disease.
Neha Nainani; Mandip Panesar
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Publication Detail:
Type:  Journal Article; Review     Date:  2008-07-29
Journal Detail:
Title:  American journal of nephrology     Volume:  29     ISSN:  1421-9670     ISO Abbreviation:  Am. J. Nephrol.     Publication Date:  2009  
Date Detail:
Created Date:  2008-10-23     Completed Date:  2008-12-03     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8109361     Medline TA:  Am J Nephrol     Country:  Switzerland    
Other Details:
Languages:  eng     Pagination:  1-9     Citation Subset:  IM    
Copyright Information:
Copyright 2008 S. Karger AG, Basel.
State University of New York at Buffalo, Buffalo, NY, USA.
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MeSH Terms
Disease Progression
Fibrosis / diagnosis*,  pathology*
Kidney Diseases / diagnosis*,  etiology,  pathology
Kidney Failure, Acute / pathology
Models, Biological
Nephrology / methods*
Nephrons / pathology*
Skin Diseases / diagnosis*,  etiology,  pathology

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