title: Kidney transplant outcomes in patients with Fabry disease.
abstract: BACKGROUND
  Fabry disease is a rare but important cause of end-stage renal disease (ESRD) among young men. Postkidney transplantation outcomes among patients with Fabry disease remain controversial.
  
  
  METHODS
  Using data from Organ Procurement Transplant Network/United Network for Organ Sharing, 197 kidney transplant recipients with ESRD because of Fabry disease from 1987 to 2007 were identified. We compared rates of graft loss and death with those of kidney transplant recipients with other (non-Fabry) causes of ESRD. Fabry patients were then compared with a 10:1 matched cohort of transplant recipients with other causes of ESRD.
  
  
  RESULTS
  Five-year graft survival was superior among Fabry patients (74%) compared with those with other causes of ESRD (69%), but was similar to those in the matched cohort (P=0.64). Five-year patient survival among Fabry patients (81%) was similar to those with other causes of ESRD (P=0.33), but was inferior to the matched cohort (90%). Cox multivariate analysis revealed that Fabry patients had a 40% lower risk of returning to dialysis compared with both matched and unmatched cohorts, but had a higher risk of death (2.15; 1.52-3.02) compared with the matched cohort.
  
  
  CONCLUSIONS
  This analysis of 197 kidney transplant recipients with Fabry indicates that they have superior graft survival and similar patient survival compared with patients with other causes of ESRD. However, Fabry patients had a higher risk of death compared with a matched cohort of patients with other causes of ESRD. This requires further investigation and may suggest a need for further attention to the minimization of cardiovascular death in this group of patients.
authors.affiliation: St. Vincent Medical Center/National Institute of Transplantation, Los Angeles, CA, USA.
authors.forename: Tariq
authors.initials: T
authors.lastname: Shah
authors.forename: Jagbir
authors.initials: J
authors.lastname: Gill
authors.forename: Neetu
authors.initials: N
authors.lastname: Malhotra
authors.forename: Steven K
authors.initials: SK
authors.lastname: Takemoto
authors.forename: Suphamai
authors.initials: S
authors.lastname: Bunnapradist
mesh_terms: D000293:Adolescent; D000328:Adult; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D000795:Fabry Disease / Q000150:complications* / Q000401:mortality / Q000601:surgery; D005260:Female; D006084:Graft Rejection / Q000209:etiology* / Q000401:mortality; D006085:Graft Survival; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D016030:Kidney Transplantation / Q000009:adverse effects / Q000401:mortality; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D013997:Time Factors; D009927:Tissue and Organ Procurement; D016896:Treatment Outcome; D014481:United States; D055815:Young Adult