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TL;DR
This is a case report of a 15-year-old male with end-stage liver disease from HNF1B disease who underwent liver transplantation and subsequently developed insulin-dependent diabetes post-transplant, which persisted after stopping steroids.
JPGN Reports · 7 authors, 4 centres
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This is a case report of a 15-year-old male with end-stage liver disease from HNF1B disease who underwent liver transplantation and subsequently developed insulin-dependent diabetes post-transplant, which persisted after stopping steroids.
This case report describes an adolescent male with HNF1B disease who presented with cholestatic liver disease in infancy, progressed to decompensated cirrhosis by age 14, and underwent liver transplantation at age 15. Pre-transplant, he had normal pancreatic endocrine function despite an atrophic-appearing pancreas. Post-transplant, while receiving prednisone, tacrolimus, and mycophenolate, he developed hyperglycemia requiring insulin. Insulin dependency persisted after steroids and mycophenolate were discontinued, with a reduction in insulin requirements from 0.88 to 0.3 units/kg/day over 12 months, though a temporary cessation failed when tacrolimus dose increased. The report highlights this as the first case of pediatric liver transplantation for decompensated cirrhosis due to HNF1B disease, with post-transplant diabetes likely related to the underlying genetic predisposition exacerbated by immunosuppression, particularly tacrolimus.