MODY (Maturity-Onset Diabetes of the Young)

What is MODY?

MODY is a rare, inherited form of diabetes caused by a single gene mutation. It is NOT Type 1 or Type 2. It is often misdiagnosed as Type 1 or Type 2.

Who should suspect MODY?

  • Diabetes diagnosed before age 25.
  • Strong family history: Diabetes in multiple generations (parent, grandparent, siblings).
  • Not overweight and no insulin resistance.
  • Negative diabetes autoantibodies (so not Type 1).

Types of MODY:

  • MODY 2 (GCK): Mild, stable high blood sugar from birth. Usually does NOT need medication. Complications are rare.
  • MODY 3 (HNF1A): Progressive. Responds extremely well to Sulfonylurea pills (like Glyburide). Insulin is rarely needed.
  • MODY 1, 4, 5, etc.: Rarer forms.

Why does the diagnosis matter?

  • Treatment is different. Some MODY types can be managed with low-dose pills instead of insulin.
  • It affects family planning (50% chance of passing it to each child).
  • Prognosis is different (some forms have minimal complications).

How is it diagnosed?

Genetic testing is required. This is not a standard test; you must specifically test for MODY genes.

Resources: