MODY Diabetes/english

Diabetes type MODY.

What is it?

This type of diabetes is named after the abbreviation of the English phrase, Maturity Onset Diabetes of the Young (middle age diabetes that occurs in the young). MODY diabetes at first may seem to type 2, which starts at maturity without insulin, but the MODY occurs at an early age, before age 25 and are not obese.

MODY diabetes is far less common, accounting for 2-5% of cases, but is often undiagnosed. It is due to a genetic problem of dominant inheritance, i.e., strongly transmitted from parents to children. There are different subtypes of MODY diabetes, dependent on the genetic problem that exists and, depending on the type, can be treated with diet, oral drugs or insulin need, especially in the long evolution.

Most cases of diabetes are due to MODY heterozygous mutations in 6 genes (4):

Gene encoding the enzyme glucokinase: MODY subtype 2 (9).
Hepatic nuclear factor 4 alpha: MODY subtype 1 (10).
Hepatic nuclear factor 1alpha: MODY subtype 3 (11).
Factor 1 promoter of insulin MODY subtype 4 (12).
Hepatic nuclear factor-1 beta: MODY subtype 5 (13).
Neurogenic differentiation factor 1, also called beta 2: MODY subtype 6 (14).

Symptoms.

Asymptomatic hyperglycemia.
It not usually associated with obesity or overweight.
Usually a slow and progressive evolution.
Deficit in insulin secretion.
It not usually starts with cetonurias.
Slow and progressive evolution.

Treatment.

The clinical perspective and treatment for MODY diabetes depends on the genetic cause. The MODY 2 can be managed with diet and exercise with excellent prognosis. Subtypes 1, 3 and 4 respond well to sulfonylureas, though a significant proportion of them (30-40% for MODY MODY 1 and 3) possibly require insulin therapy due to progressive failure of beta cells. The MODY 5 requires replacement of the endocrine and exocrine pancreatic function, and treatment for multiple organ abnormalities. In overweight patients weight loss improves hyperglycemia.

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