Neonatal diabetes with end-stage nephropathy: pancreas transplantation decision.
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.


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.
Esmatjes
Enric
E
1Endocrinology and Diabetes Unit, Hospital Clínic Universitari, Barcelona, Spain. esmatjes@clinic.ub.es
Jimenez
Amanda
A
Diaz
Gonzalo
G
Mora
Mireia
M
Casamitjana
Roser
R
Pérez de Nanclares
G
G
Castaño
Luis
L
José Ricart
Maria
M
D000328:Adult; D003922:Diabetes Mellitus, Type 1 / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D003928:Diabetic Nephropathies / Q000209:etiology / Q000628:therapy*; D005500:Follow-Up Studies; D005905:Glyburide / Q000627:therapeutic use; D006801:Humans; D007004:Hypoglycemic Agents / Q000627:therapeutic use; D007231:Infant, Newborn; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000628:therapy*; D016030:Kidney Transplantation; D008297:Male; D016035:Pancreas Transplantation; D016896:Treatment Outcome
D007004:Hypoglycemic Agents; D005905:Glyburide