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MODY (Maturity Onset Diabetes of the Young)

A monogenic form of diabetes that begins at a young age and is 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.

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

What is MODY (Maturity Onset Diabetes of the Young)?

MODY (Maturity Onset Diabetes of the Young) is the most common form of monogenic diabetes inherited in an autosomal dominant manner due to single-gene mutations affecting pancreatic beta-cell function. It usually begins before the age of 25 and accounts for approximately 1-2% of all cases of diabetes.

More than thirteen gene subtypes have been described. The most common forms are HNF1A-MODY (MODY3), GCK-MODY (MODY2) and HNF4A-MODY (MODY1). Each subtype has different clinical characteristics, diabetes course and treatment response; correct diagnosis determines individualised treatment.

GCK-MODY typically presents as mild, stable hyperglycaemia and may not require treatment. HNF1A-MODY responds excellently to sulfonylureas. It should be remembered that patients incorrectly diagnosed as type 1 diabetes and started on insulin can be treated with oral agents after genetic testing.

Symptoms

Hyperglycaemia starting at a young age (<25)
Mild-to-moderate elevation of blood glucose
Sparse classic diabetes symptoms
Strong family history of diabetes
Absence of autoantibodies and ketosis
Normal-to-slightly elevated weight
Lack of insulin resistance
Stable, slowly progressive course

Risk Factors

Multi-generational family history of diabetes
Diagnosis under 25 years of age
Hyperglycaemia without obesity
Negative pancreatic autoantibodies
Absence of ketoacidosis
Long-term beta-cell function
Family history of consanguineous marriage
Atypical diabetes course

When to See a Doctor?

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

  • Atypical diabetes diagnosed at a young age
  • Family history of diabetes at an early age
  • Lean patient not requiring insulin
  • Autoantibody-negative diabetes
  • Stable mild hyperglycaemia
  • Young patient with low insulin requirement

Treatment Methods

01
Genetic testing (definitive diagnosis)
02
Sulfonylurea treatment (HNF1A and HNF4A-MODY)
03
Lifestyle and dietary therapy (GCK-MODY)
04
Insulin (in selected forms)
05
Family screening
06
Genetic counselling
07
Long-term endocrinology follow-up
08
Pregnancy planning and management

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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MODY is a group of monogenic diabetes subtypes with autosomal dominant inheritance and onset typically before 25 years.

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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.