Maturity-onset diabetes of the young with end-stage nephropathy: A new indication for simultaneous pancreas and kidney transplantation?
Abstract
Background and Case. Simultaneous pancreas and kidney transplantation (SPK) is applied almost exclusively in C-peptide-negative type 1 diabetic patients, although some data on SPK in type 2 diabetes have been published as well.
Nothing is known about SPK in the autosomal diabetes form, maturity-onset diabetes of the young (MODY). SPK was performed in a 47-year old man who has MODY3 because of a Arg272His mutation in the hepatocyte nuclear factor-la gene.
He developed overt diabetes mellitus at 19 years and end-stage diabetic nephropathy 26 years thereafter. Before SPK, the patient had measurable fasting serum C-peptide levels, but lacked 13-cell response to intravenous glucose and glucagon.
He was treated with 34 IU of insulin per day. At 2 years posttransplantation, the patient remains normoglycemic and insulin independent.
A hyperglycemic clamp test showed a normal beta-cell function. Conclusion.
Identification of MODY3 among all C-peptide-positive patients with advanced diabetic nephropathy might help to select a specific group profiting from SPK.