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.
Department of Surgery, National Naval Medical Center, Bethesda, Maryland, USA.
A
A
Bolanowski
R B
RB
Mannon
S M
SM
Holland
H L
HL
Malech
J
J
Aschan
J
J
Palmblad
D A
DA
Hale
A D
AD
Kirk
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