From the Guidelines
MODY (Maturity-Onset Diabetes of the Young) is classified into several subtypes, including MODY 1 (HNF4A gene mutation), MODY 2 (GCK gene mutation), MODY 3 (HNF1A gene mutation), MODY 5 (HNF1B gene mutation), and others 1.
Key Characteristics of MODY Subtypes
- MODY 1 (HNF4A gene mutation): progressive insulin secretory defect with presentation in adolescence or early adulthood, may have large birth weight and transient neonatal hypoglycemia, sensitive to sulfonylureas 1
- MODY 2 (GCK gene mutation): stable, nonprogressive elevated fasting blood glucose, typically does not require treatment, microvascular complications are rare, small rise in 2-h PG level on OGTT (<54 mg/dL [3 mmol/L]) 1
- MODY 3 (HNF1A gene mutation): progressive insulin secretory defect with presentation in adolescence or early adulthood, lowered renal threshold for glucosuria, large rise in 2-h PG level on OGTT (>90 mg/dL [5 mmol/L]), sensitive to sulfonylureas 1
- MODY 5 (HNF1B gene mutation): developmental renal disease (typically cystic), genitourinary abnormalities, atrophy of the pancreas, hyperuricemia, gout 1
Treatment of MODY
Treatment for MODY varies by subtype, but may include sulfonylureas such as glyburide (5-10mg daily) or glipizide (2.5-10mg daily) for MODY 3, and metformin (500-1000mg twice daily) for MODY 2 1. Duration of treatment is typically lifelong, with regular monitoring of blood glucose levels and adjustment of medication as needed. Some subtypes, such as MODY 1 and MODY 3, are sensitive to sulfonylureas, while others, such as MODY 2, may not require treatment or may be treated with metformin 1. It's worth noting that the evidence is consistent across multiple studies, with similar classifications and treatment options reported in different sources 1.