Maturity-onset diabetes of the young with end-stage nephropathy: a new indication for simultaneous pancreas and kidney transplantation?

Research output: Contribution to journalJournal articleResearchpeer-review

Frantisek Saudek, Stepánka Pruhová, Peter Boucek, Jan Lebl, Milos Adamec, Jakob Ek, Oluf Pedersen, Torben Hansen

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-1alphagene. 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 beta-cell response to intravenous glucose and glucagon. He was treated with 34 IU of insulin per day. At 2 years post-transplantation, 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.
Original languageEnglish
JournalTransplantation
Volume77
Issue number8
Pages (from-to)1298-301
Number of pages4
ISSN0041-1337
Publication statusPublished - 2004

    Research areas

  • C-Peptide, DNA-Binding Proteins, Diabetes Mellitus, Type 2, Diabetic Nephropathies, Glucose Clamp Technique, Glucose Tolerance Test, Hepatocyte Nuclear Factor 1, Hepatocyte Nuclear Factor 1-alpha, Humans, Kidney Transplantation, Male, Middle Aged, Nuclear Proteins, Pancreas Transplantation, Point Mutation, Transcription Factors

ID: 38457404