Maturity-onset diabetes of the young with end-stage nephropathy: a new indication for simultaneous pancreas and kidney transplantation?
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Maturity-onset diabetes of the young with end-stage nephropathy : a new indication for simultaneous pancreas and kidney transplantation? / Saudek, Frantisek; Pruhová, Stepánka; Boucek, Peter; Lebl, Jan; Adamec, Milos; Ek, Jakob; Pedersen, Oluf; Hansen, Torben.
In: Transplantation, Vol. 77, No. 8, 2004, p. 1298-301.Research output: Contribution to journal › Journal article › Research › peer-review
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TY - JOUR
T1 - Maturity-onset diabetes of the young with end-stage nephropathy
T2 - a new indication for simultaneous pancreas and kidney transplantation?
AU - Saudek, Frantisek
AU - Pruhová, Stepánka
AU - Boucek, Peter
AU - Lebl, Jan
AU - Adamec, Milos
AU - Ek, Jakob
AU - Pedersen, Oluf
AU - Hansen, Torben
PY - 2004
Y1 - 2004
N2 - 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.
AB - 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.
KW - C-Peptide
KW - DNA-Binding Proteins
KW - Diabetes Mellitus, Type 2
KW - Diabetic Nephropathies
KW - Glucose Clamp Technique
KW - Glucose Tolerance Test
KW - Hepatocyte Nuclear Factor 1
KW - Hepatocyte Nuclear Factor 1-alpha
KW - Humans
KW - Kidney Transplantation
KW - Male
KW - Middle Aged
KW - Nuclear Proteins
KW - Pancreas Transplantation
KW - Point Mutation
KW - Transcription Factors
M3 - Journal article
C2 - 15114102
VL - 77
SP - 1298
EP - 1301
JO - Transplantation
JF - Transplantation
SN - 0041-1337
IS - 8
ER -
ID: 38457404