Table 1.

Clinical features of the common forms of MODY

Cardinal features suggestive of all types of MODY

Hyperglycaemia diagnosed at < 30 years of age

Nonobese and no evidence of insulin resistance

> 2 Linear generational family history of hyperglycaemia

Negative pancreatic auto-antibodies (GAD, IAA, ICA, ZnT8)

Features suggestive of GCK-MODY (MODY 2)

Stable HbA1c < 62 mmol/mol (< 7.8%)

Mild fasting hyperglyacemia from birth

Features suggestive of HNF4A/1A-MODY (MODY 1/3)

Positive serum or urine C-peptide 3–5 years after initial diagnosis [18]

Sensitivity to sulfonylurea therapy