Maturity Onset Diabetes of the Young (MODY)

This is a rare type of diabetes. Patients are non-insulin dependent. Most of them can be treated by oral hypoglycemic drugs.

Some of its Important features:

* Primary defect is impaired glucose induced secretion of insulin.

* Age is usually less than 25 years.

* This is inherited by a mechanism called autosomal dominance.

* No autoimmunity (as in type 1)

* No insulin resistance (as in type 2)

* No obesity (as in type 2)

MODY is absolutely different category. It doesn’t lead to type 2 diabetes in old age.

What are the causes of MODY?

There are six types of MODY. Except MODY 2, all are caused by mutations of nuclear transcription factor that regulates islet cell gene expression.

MODY 2 is caused by mutation of Glucokinase enzyme. Glucokinase enzyme in beta cells controls the glucose entry in to beta cells which inturn coupled with insulin release. Mutations of this enzyme increase threshold for insulin release. So insulin secretion is low for the degree of hyperglycemia.

Clinical course:

Usually mild type of diabetes occurs with slight fasting hyperglycemia and few mild symptoms of micro vascular complications. Upto 50% of carriers of glucokinase mutations develop gestational diabetes.

[tags]mody, diabetes, diabetic[/tags]