title: Dipeptidyl peptidase-IV inhibitors are efficient adjunct therapy in HNF1A maturity-onset diabetes of the young patients--report of two cases.
abstract: BACKGROUND
  In HNF1A maturity-onset diabetes of the young (MODY), sulfonylurea (SU) is the first-line treatment. Over time, such therapy fails, and additional treatment is required. Dipeptidyl peptidase IV (DPP-IV) inhibitors are new agents that lower blood glucose by prolonging the activity of circulating incretins.
  
  
  METHODS
  We applied DPP-IV inhibitors in two HNF1A MODY patients whose earlier therapeutic regimen included SU.
  
  
  RESULTS
  Case 1, a 39-year-old woman, a carrier of the ArgR171X HNF1A mutation, with a 7-year history of diabetes was on 160 mg of gliclazide and 2,000 mg of metformin. Her initial hemoglobin A1c (HbA1c) level was 7.2%, while the mean glucose level on the CGMS((R)) (Medtronic, Northridge, CA) record was 162 mg/dL. Sitagliptine, in a dose of 100 mg/day, was added to the previous treatment. Case 2, a 62-year-old woman, a carrier of the IVS7nt-6G>A mutation, with a 41-year history of diabetes was treated with 240 mg/day gliclazide and 6 IU of insulin/day. Her initial HbA1c was 8.8%, and average glycemia reached 172 mg/dL. In her case, we started the combined therapy with 50 mg of vildagliptine twice daily. Patients were reexamined after 3 months, and HbA1c fell to 6.3% in both subjects. Similarly, significant improvement in glycemic control on CGMS was observed as the average glycemia decreased to 114 mg/dL and 134 mg/dL in Case 1 and Case 2, respectively. No episodes of hypoglycemia or other side effects were recorded. As intravenous glucose tolerance tests (IVGTTs) were performed before and after DPP-IV implementation, we were able to assess their impact on insulin secretion under fasting conditions. We saw a substantial rise in insulin level increment during IVGTT (by 9.8 and13.4 mIU/L in Case 1 and Case 2, respectively).
  
  
  CONCLUSIONS
  DPP-IV inhibitors may be an effective tool of combined therapy in HNF1A MODY, and they seem to improve beta-cell function under fasting conditions.
authors.affiliation: Department of Metabolic Diseases, Jagiellonian University Medical College, 15 Kopernika Street, Krakow, Poland.
authors.forename: Barbara
authors.initials: B
authors.lastname: Katra
authors.forename: Tomasz
authors.initials: T
authors.lastname: Klupa
authors.forename: Jan
authors.initials: J
authors.lastname: Skupien
authors.forename: Magdalena
authors.initials: M
authors.lastname: Szopa
authors.forename: Natalia
authors.initials: N
authors.lastname: Nowak
authors.forename: Maciej
authors.initials: M
authors.lastname: Borowiec
authors.forename: Elzbieta
authors.initials: E
authors.lastname: Kozek
authors.forename: Maciej T
authors.initials: MT
authors.lastname: Malecki
mesh_terms: D000328:Adult; D017668:Age of Onset; D003920:Diabetes Mellitus / Q000188:drug therapy* / Q000235:genetics; D054873:Dipeptidyl-Peptidase IV Inhibitors / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D005951:Glucose Tolerance Test; D051538:Hepatocyte Nuclear Factor 1-alpha / Q000235:genetics*; D006801:Humans; D007004:Hypoglycemic Agents / Q000627:therapeutic use; D008875:Middle Aged
chemical_list: D054873:Dipeptidyl-Peptidase IV Inhibitors; C496487:HNF1A protein, human; D051538:Hepatocyte Nuclear Factor 1-alpha; D007004:Hypoglycemic Agents