Successful renal transplantation in patients with chronic granulomatous disease

Am J Transplant. 2006 Mar;6(3):636-9. doi: 10.1111/j.1600-6143.2006.01232.x.

Abstract

Chronic granulomatous disease (CGD) is a genetic disease caused by structural mutations in the enzyme NADPH oxidase that results in severe immunodeficiency. End-stage renal disease occurs in this patient population, and is often attributed to the necessary use of nephrotoxic anti-infectives. In this report, we present the experiences of two centers in transplantation of three patients with CGD: one transplanted with CGD, one cured of his CGD with bone marrow transplantation who subsequently underwent kidney transplantation and one that received a kidney transplant prior to being cured of CGD via a sequential peripheral blood stem cell transplant (SCT). All three recipients have enjoyed excellent outcomes. Their courses demonstrate the absolute requirements for a multidisciplinary and compulsive approach before, during and after transplantation. These case reports also highlight the unexpectedly benign effects of immunosuppressive therapy in this patient population.

Publication types

  • Case Reports
  • Research Support, N.I.H., Intramural

MeSH terms

  • Adult
  • Child
  • DNA / genetics
  • Female
  • Follow-Up Studies
  • Granulomatous Disease, Chronic / enzymology
  • Granulomatous Disease, Chronic / genetics
  • Granulomatous Disease, Chronic / surgery*
  • Humans
  • Kidney Transplantation*
  • Male
  • Mutation
  • NADPH Oxidases / genetics
  • Treatment Outcome

Substances

  • DNA
  • NADPH Oxidases