Table 2:

Clinical and laboratory features of HNF1A diabetes (maturity-onset diabetes of the young type 3)3,1318

Clinical considerationsLaboratory features
Autosomal dominant inheritance; often more than three generations with diabetesC- peptide detectable
High penetrance in familiesIslet cell antibodies usually absent3
European ethnicity most commonNormal triglyceride levels
Lean (BMI often < 25)Normal or high HDL cholesterol levels 17
Usually young (< 25 yr)Low C-reactive protein levels with high-sensitivity testing18
No ketoacidosisLow renal threshold for glucose; glucosuria often occurs when serum glucose > 8 mmol/L14
Increased insulin sensitivity; patients often have minimal requirement for insulin14Large (> 3.0 mmol/L) rise in 2-h glucose levels on 75-g oral glucose tolerance testing13
Hypersensitivity to sulfonylurea drugs15For up to 50% of patients, fasting glucose < 5.5 mmol/L early in disease course13
Postprandial hyperglycemia dominates
Prevalence of microvascular and macrovascular complications similar to that of patients with type 1 diabetes16
Progressive β-cell failure over time, with increased fasting glucose13
  • Note: BMI = body mass index, HDL = high-density lipoprotein, HNF1A = hepatocyte nuclear factor 1α.