En bloc multiorgan transplant (liver, pancreas, and kidney) for acute liver and renal failure in a patient with wolcott rallison syndrome
Wolcott Rallison syndrome (WRS) is a rare genetic disease, with a known poor prognosis. Its main clinical features are infantile onset insulin dependent diabetes mellitus, neutropenias, recurrent infections, evolving bone dyscrasias, multiple epiphyseal dysplasias, renal failure, and recurrent acute liver failure secondary to stressors such as common viral illnesses. Liver failure appears to be a characteristic feature of WRS, and most children die before they turn 10 years old. In the study by Habeb et al. all patients died from liver failure instigated by a flu like illness. En bloc transplantation of the liver, pancreas, and kidney is a technically challenging procedure, but it is the most effective option to manage complications associated with WRS and increase patient survival. Currently, except for 1 prior report, the available evidence in the literature is insufficient to discern if patients who present with acute liver and renal failure secondary to WRS should be transplanted or not.
Liver transplantation official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society