Liver, Pancreas and Kidney Transplantation for the Treatment of Wolcott–Rallison Syndrome

    loading  Checking for direct PDF access through Ovid

Abstract

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.

This report illustrates the first case presenting the utilization of en bloc multi-organ transplantation to reduce the complications of Wolcott-Rallison syndrome.

Related Topics

    loading  Loading Related Articles