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]


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.


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.


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]


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]

< Prev   Next >

Common Diseases

Swine Flu - Updates and information on H1N1 2009 (AKA Swine Influenza) pandemic.

Ankylosing spondylitis - Current protocols for diagnosis and treatment options.

Wegener granulomatosis - Autoimmune etiology and clinical course.

Diabetes - disease and management information, including diagnosis, typical treatment plans and diabetes supplies.


Medical Careers

The US medical jobs market has stayed hot for health care providers. Whether you believe that a provider shortage is in the offing or that the ratio of physicians-to-patients is too high, physician jobs and nursing jobs abound.

A wide variety of medical jobs can be found in the netdoc health care job listings. Particular strengths include permanent and locum tenens physician jobs, nursing jobs across the US, and radiology positions.

Other resources include physician salary information, medical career guidance, and the ability to post physician jobs.


When hiring your medical practice office manager, what was the most important consideration?
Copyright 2005 - 2019 Medical Resource Group, LLC. All rights reserved.