Table 3 Clinical criteria suggesting diagnosis of HNF1A-MODY

Familial history of diabetes (at least two generations)

Young-onset diabetes (typically before age 25 in at least one family member)

Incomplete insulin-dependency outside the normal honeymoon period (3 years) as demonstrated by:

 · Not developing ketoacidosis in the absence of insulin;

 · Good glycemic control on less than the usual replacement dose of insulin, or;

 · Detectable C-peptide measured when on insulin with glucose > 8 mmol/l (140 mg/dL)

Glucose increment usually > 5 mmol/l (90 mg/dL) in an OGTT (normal fasting values with 2-hour values in the diabetic range are common and a useful feature to contrast with GCK)

Absence of pancreatic islet autoantibodies

Glycosuria at blood glucose levels < 10 mmol/L (180 mg/dL), due to a low renal glucose reabsorption threshold

Marked sensitivity to sulfonylureas (resulting in hypoglycemia despite poor glycemic control before transitioning to secretagogue agents)

Absence of characteristics of insulin resistance that could suggest type 2 diabetes rather than monogenic diabetes, such as obesity, acanthosis nigricans, and belonging to ethnic groups at risk for type 2 diabetes