Successful renal transplantation in patients with chronic granulomatous disease.
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.
Bolanowski
A
A
Department of Surgery, National Naval Medical Center, Bethesda, Maryland, USA.
Mannon
R B
RB
Holland
S M
SM
Malech
H L
HL
Aschan
J
J
Palmblad
J
J
Hale
D A
DA
Kirk
A D
AD
D000328:Adult; D002648:Child; D004247:DNA / Q000235:genetics; D005260:Female; D005500:Follow-Up Studies; D006105:Granulomatous Disease, Chronic / Q000201:enzymology / Q000235:genetics / Q000601:surgery*; D006801:Humans; D016030:Kidney Transplantation; D008297:Male; D009154:Mutation; D019255:NADPH Oxidases / Q000235:genetics; D016896:Treatment Outcome
D004247:DNA; D019255:NADPH Oxidases