title: Neonatal diabetes with end-stage nephropathy: pancreas transplantation decision.
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.
  
  
  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.
authors.affiliation: 1Endocrinology and Diabetes Unit, Hospital Clínic Universitari, Barcelona, Spain. esmatjes@clinic.ub.es
authors.forename: Enric
authors.initials: E
authors.lastname: Esmatjes
authors.forename: Amanda
authors.initials: A
authors.lastname: Jimenez
authors.forename: Gonzalo
authors.initials: G
authors.lastname: Diaz
authors.forename: Mireia
authors.initials: M
authors.lastname: Mora
authors.forename: Roser
authors.initials: R
authors.lastname: Casamitjana
authors.forename: G
authors.initials: G
authors.lastname: Pérez de Nanclares
authors.forename: Luis
authors.initials: L
authors.lastname: Castaño
authors.forename: Maria
authors.initials: M
authors.lastname: José Ricart
mesh_terms: 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
chemical_list: D007004:Hypoglycemic Agents; D005905:Glyburide