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MODY syndrome

Orpha number ORPHA552
Synonym(s) Maturity-onset diabetes of the young
Prevalence Unknown
Inheritance
  • Autosomal dominant
Age of onset Adulthood
ICD-10
  • E11.8
OMIM
UMLS -
MeSH -
MedDRA -
SNOMED CT
  • 28453007

Summary

MODY syndrome (Maturity Onset Diabetes of the Young) is a rare, familial, clinically and genetically heterogeneous form of young age-onset diabetes characterized by often mild diabetes, lack of pancreatic beta-cell autoimmunity, absence of obesity, and extra-pancreatic manifestations in some patients.


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Detailed information

Guidance for genetic testing
  • EN (2014,pdf)
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