Criteria for Maturity-Onset Diabetes Associated with Anomalies of HNF1A (MODY 3)

 

Gene affected: hepatocyte nuclear factor-1alpha (HNF1A)

 

Inheritance: autosomal dominant

 

Reasons to suspect MODY3:

(1) age of the patient at onset < 25 years (although an older age does not exclude the diagnosis)

(2) number of affected generations with diabetes >= 3

(3) lower BMI AND absence of diabetic symptoms

(4) Euro-Caucasian descent

 

Exclusions:

(1) autoimmune diabetes

(2) insulin-dependent diabetes

 

Definitive diagnosis involves assessment of the HNF1A gene.