Liver, pancreas and kidney transplantation for the treatment of Wolcott-Rallison syndrome.
We present the case of a child who underwent a combined liver, pancreas and double kidney transplant following complications of Wolcott-Rallison syndrome (WRS) a rare genetic disorder that causes infantile insulin-dependent diabetes mellitus (IDDM) and often death in childhood from fulminant liver and concomitant kidney failure. WRS is characterized clinically through infantile IDDM, propensity for liver failure following viral infections, bone dysplasia and growth failure and developmental delay. Fewer than 60 cases with WRS are reported in the literature, mostly from consanguineous parents. Future episodes of liver failure, the main contributor to the increased mortality in WRS, may be prevented through timely liver transplantation. To the best of our knowledge, transplantation has not been utilized to manage complications of WRS prior to this report.
Transplant Center, Cleveland Clinic Florida, Weston, FL.
A G
AG
Tzakis
M J
MJ
Nunnelley
A
A
Tekin
L D
LD
Buccini
J
J
Garcia
K
K
Uchida
H L
HL
Neville
M A
MA
Nares
P
P
Ruiz
O
O
Bodamer
D002648:Child; D003922:Diabetes Mellitus, Type 1 / Q000150:complications / Q000453:epidemiology / Q000601:surgery*; D004838:Epiphyses / Q000002:abnormalities* / Q000601:surgery; D005260:Female; D006801:Humans; D016030:Kidney Transplantation; D017114:Liver Failure, Acute / Q000453:epidemiology; D016031:Liver Transplantation; D010009:Osteochondrodysplasias / Q000150:complications / Q000601:surgery*; D016035:Pancreas Transplantation; D051437:Renal Insufficiency / Q000453:epidemiology; D012307:Risk Factors; D016896:Treatment Outcome
clinical research/practice; kidney transplantation/nephrology; liver transplantation/hepatology; pediatrics; quality of care/care delivery; surgical technique