Monogenic diabetes is a family of rare genetic conditions that arise from a mutation on a single gene. These conditions are usually diagnosed in young people (25 or younger) and often run in families.
MODY
The best known form of monogenic diabetes is maturity-onset diabetes of the young (MODY). This condition is distinct from types 1 and 2 diabetes, and doesn’t involve autoimmunity or insulin resistance, but can be misdiagnosed as either. MODY typically does not require insulin and is usually managed with other glucose-lowering drugs such as sulfonylureas, though the precise treatment of MODY will differ depending on which gene is affected.
If a parent has MODY, their child has a 50 percent risk of developing the condition. Genetic testing is key to obtaining an accurate diagnosis.
Neonatal Diabetes
Neonatal diabetes is a rare form of monogenic diabetes that develops within the first six months of a child’s life. Unlike type 1 diabetes, it’s not caused by the immune system. An accurate neonatal diabetes diagnosis requires genetic testing.
Neonatal diabetes can be transient, which means it can resolve by itself, though it may possibly return later in life. It can also be permanent, requiring daily insulin therapy indefinitely.
Signs and symptoms of neonatal diabetes include inadequate weight gain, dehydration, and high blood sugar. The earlier neonatal diabetes is diagnosed, the more likely long-term complications can be prevented.
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