Unexpected fabry disease in a renal allograft kidney: An underrecognized cause of poor allograft function

Olga Kochar, Mark R. Wick, Sarah E. Kerr, Devin Oglesbee, Helen P. Cathro

Research output: Contribution to journalArticle

7 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)92-96
Number of pages5
JournalUltrastructural Pathology
Volume35
Issue number2
DOIs
StatePublished - Apr 1 2011

Keywords

  • Allograft biopsy
  • Deceased donor
  • Fabry disease
  • Myelin-like inclusions

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Structural Biology

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