title: Long-term response to sulfonylurea in a patient with diabetes due to mutation in the KCNJ11 gene.
abstract: OBJECTIVE
  To report the long-term (30-month) effect of the switch from insulin to sulfonylurea in a patient carrying the p.G53D (c.158G>A) mutation in KCNJ11 gene.
  
  
  METHODS
  A 29-year-old male patient was diagnosed with diabetes in the third month of life and after identification of a heterozygous p.G53D mutation in the KCNJ11 gene, the therapy was switched from insulin to sulfonylurea.
  
  
  RESULTS
  Long-term follow-up (30 months) showed that good metabolic control was maintained (HbA1c: 6.6%) and the glibenclamide dose could be reduced.
  
  
  CONCLUSIONS
  Long-term therapy with sulfonylureas in patients with neonatal diabetes due to mutation in the KCNJ11 gene is safe and promotes sustained improvement of glycemic control.
authors.affiliation: Division of Endocrinology, Universidade Federal de São Paulo, SP, Brazil.
authors.forename: Marcio F
authors.initials: MF
authors.lastname: Vendramini
authors.forename: Lucimary C
authors.initials: LC
authors.lastname: Gurgel
authors.forename: Regina S
authors.initials: RS
authors.lastname: Moisés
mesh_terms: D000328:Adult; D003920:Diabetes Mellitus / Q000188:drug therapy* / Q000235:genetics / Q000378:metabolism; D057915:Drug Substitution; D006579:Heterozygote; D006801:Humans; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases / Q000188:drug therapy* / Q000235:genetics; D008297:Male; D009154:Mutation / Q000187:drug effects* / Q000235:genetics; D024661:Potassium Channels, Inwardly Rectifying / Q000187:drug effects* / Q000235:genetics; D013453:Sulfonylurea Compounds / Q000627:therapeutic use*; D016896:Treatment Outcome; D000099161:Kcnj11 Channel
chemical_list: D024661:Potassium Channels, Inwardly Rectifying; D013453:Sulfonylurea Compounds; D000099161:Kcnj11 Channel