title: Review on monogenic diabetes.
abstract: OBJECTIVE
  The goal of this review is to provide an update on the different forms of monogenic diabetes, including maturity-onset diabetes of the young (MODY) and neonatal diabetes (permanent and transient neonatal diabetes).
  
  
  RESULTS
  Monogenic diabetes accounts for approximately 1-2% of diabetes cases and results from mutations that primarily reduce β-cell function. Individuals with islet autoantibody negative youth-onset forms of diabetes should be evaluated for either glucokinase-MODY or transcription factors MODY. The mild-fasting hyperglycemia found in glucokinase-MODY typically does not necessitate pharmacological treatment, whereas patients with MODY caused by transcription factor mutations can often be successfully treated with low-dose sulfonylurea. Neonatal diabetes is defined as diabetes onset within the first 6 months of life and most individuals with permanent neonatal diabetes can be treated with high-dose sulfonylurea.
  
  
  CONCLUSIONS
  The discovery of the genetic cause of monogenic diabetes has greatly advanced our understanding and management of these uncommon forms of diabetes.
authors.affiliation: Barbara Davis Center for Childhood Diabetes, University of Colorado Denver, Aurora, Colorado 80045-6511, USA.
authors.forename: Andrea K
authors.initials: AK
authors.lastname: Steck
authors.forename: Wiliam E
authors.initials: WE
authors.lastname: Winter
mesh_terms: D000818:Animals; D003922:Diabetes Mellitus, Type 1 / Q000235:genetics* / Q000276:immunology / Q000378:metabolism; D005838:Genotype; D005941:Glucokinase / Q000235:genetics; D015803:HLA-DR3 Antigen / Q000235:genetics / Q000378:metabolism; D015804:HLA-DR4 Antigen / Q000235:genetics / Q000378:metabolism; D006801:Humans; D050417:Insulin-Secreting Cells / Q000378:metabolism / Q000473:pathology; D010641:Phenotype; D014157:Transcription Factors / Q000235:genetics
chemical_list: D015803:HLA-DR3 Antigen; D015804:HLA-DR4 Antigen; D014157:Transcription Factors; D005941:Glucokinase