Table 3 Clinical criteria suggesting diagnosis of HNF1A-MODY
From: Update on clinical screening of maturity-onset diabetes of the young (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 |