Document Detail


Unexpected fabry disease in a renal allograft kidney: an underrecognized cause of poor allograft function.
MedLine Citation:
PMID:  21299350     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Fabry disease is an X-linked recessive lysosomal storage disease caused by a deficiency of ?-galactosidase A, with characteristic ultrastructural cytoplasmic myelin-like inclusions. Renal lesions are seen in male and variably in heterozygous female patients. One previous report has described Fabry disease involving a renal allograft from a deceased female donor with no history of Fabry disease. The authors describe another case, in which suspicion for Fabry disease was raised ultrastructurally. This serves as a reminder that proteinuria after renal transplantation may be due to donor-derived disease. Fabry disease is probably an underrecognized cause of graft dysfunction. This case provides further justification for ultrastructural examination of renal allograft biopsies.
Authors:
Olga Kochar; Mark R Wick; Sarah E Kerr; Devin Oglesbee; Helen P Cathro
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Ultrastructural pathology     Volume:  35     ISSN:  1521-0758     ISO Abbreviation:  Ultrastruct Pathol     Publication Date:  2011 Apr 
Date Detail:
Created Date:  2011-02-08     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8002867     Medline TA:  Ultrastruct Pathol     Country:  England    
Other Details:
Languages:  eng     Pagination:  92-6     Citation Subset:  IM    
Affiliation:
Department of Pathology and Laboratory Medicine, University of Virginia, Charlottesville, Virginia, USA.
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