Neonatal Diabetes With End-Stage Nephropathy
Pancreas transplantation decision
- Enric Esmatjes, MD, PHD123,
- Amanda Jimenez, MD1,
- Gonzalo Diaz, MD1,
- Mireia Mora, MD1,
- Roser Casamitjana, PHD234,
- G. Pérez de Nanclares, PHD35,
- Luis Castaño, MD, PHD35 and
- Maria José Ricart, MD, PHD6
- 1Endocrinology and Diabetes Unit, Hospital Clínic Universitari, Barcelona, Spain
- 2Institut d’Investigancions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain
- 3Centro de Investigación Biomédica en Red de Diabetes y Enfermedades Metabólicas Asociadas (CIBERDEM), Barcelona, Spain
- 4Hormonal Laboratory, Hospital Clínic Universitari, Barcelona, Spain
- 5Endocrinology and Diabetes Research Group, Hospital de Cruces, Barakaldo, Basque Country, Spain
- 6Renal Transplant Unit, Hospital Clínic Universitari, Barcelona, Spain
- Corresponding author: Enric Esmatjes, esmatjes{at}clinic.ub.es
Abstract
OBJECTIVE—To describe the diagnosis of a patient with neonatal diabetes who had been misdiagnosed with type 1 diabetes and referred to our hospital for pancreas and kidney transplantation because of end-stage renal disease.
RESEARCH DESIGN AND METHODS—A diagnosis of neonatal diabetes was made after a molecular genetic study revealed a mutation in exon 34 of the ABCC8 gene. Pancreas transplantation was ill-advised.
RESULTS—The patient was switched from insulin to glibenclamide 4 months after kidney transplantation, confirming that pancreas transplantation would not have been a good decision.
CONCLUSIONS—This is the first report of a patient with neonatal diabetes who developed diabetic nephropathy that progressed to end-stage renal disease. This report illustrates that careful endocrinological evaluation, including molecular genetic studies, if necessary, is mandatory before a decision to perform a pancreas transplant is made.
Footnotes
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Published ahead of print at http://care.diabetesjournals.org on 4 August 2008.
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- Accepted July 24, 2008.
- Received May 2, 2008.
- DIABETES CARE
