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[maineman]

Anyone want a copy of the entire article, please let me know. Here's the key excerpt:

The term maturity-onset diabetes of the young was based on the old classification of diabetes into juvenile-onset and maturity-onset diabetes. The American Diabetes Association and the World Health Organization have introduced a revised, cause-based classification for diabetes. MODY is now defined as a genetic defect in β-cell function with subclassification according to the gene involved.

MODY is a familial form of non–insulin dependent diabetes with autosomal dominant inheritance that usually develops at childhood, adolescence, or young adulthood, and presents primary insulin-secretion defects. The main diagnostic criteria for MODY are
• Early onset of diabetes, frequently diagnosed before age 25 years in at least one and ideally two family members. Anticipation or progressive reduction in the age of diagnosis in succeeding generations was reported in almost all reported MODY pedigrees, probably because of enhanced awareness of diabetes leading to earlier testing.
• Non–insulin dependence shown by absence of insulin treatment 5 years after diagnosis or significant C-peptide even in a patient on insulin treatment.
• Autosomal dominant inheritance (ie, vertical transmission of diabetes through at least two or ideally three generations, with a similar phenotype in diabetic family members).
• Rare association with obesity with the MODY phenotype (obesity not required for the development of this form of diabetes).
• Diabetes resulting from β-cell dysfunction. Insulin levels are often in the normal range, though inappropriately low for the degree of hyperglycemia.
The prevalence of MODY is estimated at less than 5% of patients who have T2D in most white populations.

Taken from:
Endocrinology and Metabolism Clinics
Volume 35 • Number 2 • June 2006
Written by:
Martine Vaxillaire, PharmD, PhD
Philippe Froguel, MD, PhD