Maturity-Onset Diabetes of the Young (MODY)

Maturity-Onset Diabetes of the Young (MODY)

Maturity-Onset Diabetes of the Young (MODY) is a genetically inherited, rare form of diabetes that usually occurs before age 25. It is caused by a defect in a single gene and is diagnosed by diabetes and genetic testing. It is often mistaken for type 1 or type 2 diabetes. MODY accounts for only one to five percent of diabetes cases.

Different types of MODY

• HNF1-alpha. Seen in about 70 per cent of cases of MODY. The gene lowers the amount of insulin made by the pancreas. Insulin is usually not needed and it is treated with sulphonylurea oral medications (often used in type 2 diabetes).
• HNF4-alpha. Not very common. May have had a birth weight of 9lb or more (around 4kg) and may have needed treatment for low glucose levels soon after birth. Treatment is sulphonylurea tablets and sometimes insulin is needed.
• HNF1-beta. This type of MODY is associated with other problems such as renal cysts, abnormalities of the uterus, gout, and diabetes. Often detected before birth with renal cysts found on scans. Insulin is usually required.
• Glucokinase. Glucokinase is a gene that detects how high the blood glucose levels are in the body. If this gene malfunctions, glucose levels can be slightly higher than normal. Treatment is usually not necessary.