Neonatal diabetes with end-stage nephropathy: pancreas transplantation decision

Abstract

Objective: We describe a patient with a suggestive story of neonatal diabetes (ND) who had been erroneously considered as having type 1 diabetes and was referred to our hospital for pancreas and kidney transplantation because of end-stage renal disease.

Methods: After a molecular genetic study, the diagnosis of ND, due to a mutation in exon 34 of the ABCC8 gene, was made and pancreas transplantation was ill-advised.

Results: Four months after kidney transplantation the patient was switched from insulin to glibenclamide confirming that pancreas transplantation would not have been a good decision.

Conclusions: This is the first description of a patient with ND who developed diabetic nephropathy which progressed to end-stage renal disease and illustrates that careful endocrinological evaluation, including molecular genetic studies if necessary, of candidates for pancreas transplantation is mandatory before a decision to transplant is made.