Unexpected Fabry disease in a renal allograft kidney: an underrecognized cause of poor allograft function.

Abstract:

:Fabry disease is an X-linked recessive lysosomal storage disease caused by a deficiency of α-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.

journal_name

Ultrastruct Pathol

authors

Kochar O,Wick MR,Kerr SE,Oglesbee D,Cathro HP

doi

10.3109/01913123.2010.538136

subject

Has Abstract

pub_date

2011-04-01 00:00:00

pages

92-6

issue

2

eissn

0191-3123

issn

1521-0758

journal_volume

35

pub_type

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