title: Reevaluation of a case of type 1 diabetes mellitus diagnosed before 6 months of age.
abstract: BACKGROUND
  A 17-year-old female was referred for the reassessment of her type 1 diabetes mellitus, with which she had been diagnosed at the age of 15 weeks owing to symptoms of ketoacidosis. The patient had mild learning difficulties, which resulted in her requiring additional support at school. There was no family history of diabetes.
  
  
  METHODS
  Measurements of plasma C-peptide and glutamate decarboxylase autoantibodies. Molecular genetic testing was performed.
  
  
  METHODS
  Intermediate developmental delay, epilepsy and neonatal diabetes mellitus (DEND) syndrome as a result of a 59V>M Kir6.2 mutation.
  
  
  RESULTS
  Treatment with high-dose oral glibenclamide replaced insulin treatment. Good glycemic control was achieved with levels of HbA(1c) consistently below 6.5% and no hypoglycemia.
authors.affiliation: Peninsula Medical School, Barrack Road, Exeter EX2 5DW, UK.
authors.forename: Angus G
authors.initials: AG
authors.lastname: Jones
authors.forename: Andrew T
authors.initials: AT
authors.lastname: Hattersley
mesh_terms: D000284:Administration, Oral; D000293:Adolescent; D019943:Amino Acid Substitution; D002658:Developmental Disabilities / Q000209:etiology; D003922:Diabetes Mellitus, Type 1 / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics; D004305:Dose-Response Relationship, Drug; D004827:Epilepsy / Q000209:etiology; D005260:Female; D005905:Glyburide / Q000008:administration & dosage; D006442:Glycated Hemoglobin / Q000378:metabolism; D006801:Humans; D007004:Hypoglycemic Agents / Q000008:administration & dosage; D007223:Infant; D008715:Methionine; D009154:Mutation; D024661:Potassium Channels, Inwardly Rectifying / Q000235:genetics; D013577:Syndrome; D014633:Valine; D000099161:Kcnj11 Channel
chemical_list: D005905:Glyburide; D006442:Glycated Hemoglobin; D007004:Hypoglycemic Agents; D008715:Methionine; D024661:Potassium Channels, Inwardly Rectifying; D014633:Valine; D000099161:Kcnj11 Channel