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Monogenic Diabetes (MODY)

Inherited forms of diabetes due to single-gene mutations.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Internal Medicine department. Book Appointment →

What is Monogenic Diabetes (MODY)?

Maturity-Onset Diabetes of the Young (MODY) refers to autosomal dominant inherited forms of diabetes caused by single-gene mutations. It accounts for 1-5% of all diabetes cases but is often misdiagnosed as type 1 or type 2 diabetes.

The most common MODY types are: MODY 2 (GCK mutation - mild fasting hyperglycemia, treatment usually unnecessary), MODY 3 (HNF1A mutation - very sensitive to sulfonylureas, controlled with low doses), and MODY 1 (HNF4A mutation - similar to MODY 3).

Conditions that should raise suspicion for MODY: diabetes diagnosis under age 25, prominent family history (diabetes in at least 2 generations), negative anti-GAD and anti-IA2, measurable C-peptide level, and absence of obesity. Genetic testing provides definitive diagnosis and determines treatment.

Symptoms

Diagnosis of diabetes at a young age (<25 years)
Family history of diabetes in at least 2 generations
Mild or moderate hyperglycemia (in MODY 2)
Low or absent insulin requirement
Diabetic ketoacidosis is rare

Risk Factors

Family history of autosomal dominant diabetes
Diagnosis of diabetes at a young age
Negative islet autoantibodies
Normal or mildly increased BMI

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • When diabetes is diagnosed under the age of 25
  • When there is diabetes in more than one generation in the family
  • If C-peptide is at a measurable level in a patient diagnosed with type 1 diabetes
  • MODY should be considered if insulin doses are lower than expected

Treatment Methods

01
MODY 2 (GCK): Generally no treatment needed (except during pregnancy)
02
MODY 3 (HNF1A): Low-dose sulfonylurea (instead of insulin)
03
MODY 1 (HNF4A): Sulfonylurea (similar approach to MODY 3)
04
Genetic counseling and family screening
05
Correction of misdiagnosis (insulin discontinuation, transition to appropriate treatment)

Which Department to Visit?

You can visit our Endokrinoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.