Maturity onset diabetes of the young ( MODY )

Maturity onset diabetes of the young (MODY), is a complex group of disorders characterized by certain autosomal inherited form of diabetes. This non – insulin dependent diabetes usually occurs in young adults before the age of 25.

It is different from type 1 and type 2 diabetes and is known as monogenic diabetes. One of the major feature of MODY is that diet control and tablets are the best to treat and insulin can be avoided.

1% – 2% of all diabetes cases are MODY. Mutation in parents has a probability of 50 % to inherit MODY to their kids. Inherited offspring will surely develop MODY irrespective of race, lifestyle and medication.

Cause of MODY

Mutation is the major cause of MODY. Mutation in HNF1A (hepatocyte nuclear factor 1 alpha), HNF4A (hepatocyte nuclear factor 4 alpha) and glucokinase (GCK) are the most common and accounts for about 52%, 10% and 32% of all MODY cases.

genetics of mody

Refer: Genes responsible for MODY.

 

Clinical presentation of MODY

Clinical presentation depends on the mutation of the gene responsible for mutation. Clinical presentation can vary.

  • Certain forms of MODY will produce severe hyperglycemia and they will show typical signs and symptoms of diabetes.
  • But, certain forms of MODY have no significant signs and symptoms but are diagnosed accidently when being admitted for some other reasons. It is also found out when close relatives are monitored for diabetes.
  • Absence of certain autoantibodies to counter pancreatic antigens
  • Presence of elevate C peptide in hyperglycemia.

Diagnosis

A five step diagnostic criteria is followed:

  1. Hyperglycemia is identified before the age of 25 in 2 family members.
  2. Vertical transmission of diabetes in about 3 generations
  3. Elevated C peptide level in patients on insulin treatment
  4. Insulin levels usually ranges within normal limits suggesting beta cell dysfunction.
  5. Obesity or overweight

Refer: genetic testing to diagnose MODY.

 

 

Management of MODY

The goals of management and lifestyle modification are similar to other forms of diabetes. But, differential diagnosis may help to:

  1. Stop administering insulin and to switch from insulin to oral hypoglycemic. Here the glycemic control is not lost.
  2. Appropriate treatment can be started.
  3. It can lead to screening of relatives and to identify the same in them.

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