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Maturity onset diabetes of the young
Written by Jeffrey R. Waggoner, MD   

Maturity-onset diabetes of the young (MODY) is a condition that affects between 1% and 2% of all diabetics. It is defined by 3 characteristics:

1. it often develops prior to the age of 25

2. it is passed from one member of a family into the next generation

3. it is usually controlled with diet, oral hypoglycemics or both but does not often require exogenous insulin.

Genetic basis of maturity-onset diabetes of the young

It appears that MODY can be caused by a single defective gene. It follows a pattern of autosomal dominance. There have been 6 genes associated with the disease: HNF1-α, glucokinase, HNF1-β, HNF4-α, IPF1, Neuro D1. [1]

Clinical presentation varies with defective gene

The clinical course for four of the mutations have been identified, but the other genetic abnormalities are poorly understood. In addition, 13% of patients who have MODY clinically have not had a genetic abnormality identified. [2]

HNF1-α MODY

This form of the disease is associated with microvascular complications, both to the retina and kidney, and tends to worsen with age. Its onset is usually in adolescence or by mid-twenties, although some patients may not become diabetic until middle age. 96% of patients are typically diagnosed by age 55. Patients with HNF1-α MODY respond exceedingly well to sulfonylureas.

There is often development of profound hyperglycemia following puberty in patients with HNF1-α MODY. This can lead to an incorrect diagnosis of Type 1 diabetes. However, even during these spikes in glucose levels, a significant degree of sulfonylurea sensitivity persists.

Glucokinase

Along with HNF1-α, this is the most common of of the genetic abnormalities responsible for MODY. These patients have mild, usually asymptomatic, stable hyperglycemia from birth. They rarely require pharmacological intervention.

HNF-1β

This genetic abnormality is associated with very mild Type 2 diabetes as well as renal cysts and abnormalities of the pancreas and genital tract. [3]

HNF4-α

This genetic abnormality is poorly understood. It is associated with a very mild form of adult onset diabetes. One factor recently recognized is that an HNF4-α abnormality may occur in combination with HNF1-α abnormalities—suggesting a “double hit” theory causing MODY. [4]

 
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