<unk>  is the final diagnosis in 1%–2% of people initially diagnosed with diabetes. The prevalence is 70–110 per million people. 50% of first-degree relatives will inherit the same mutation, giving them a greater than 95% lifetime risk of developing<unk>  themselves. For this reason, correct diagnosis of this condition is important. Typically patients present with a strong family history of diabetes (any type) and the<unk>  of symptoms is in the second to fifth decade. There are two general types of clinical presentation. * Some forms of<unk>  produce significant hyperglycemia and the typical signs and symptoms of diabetes: increased thirst and urination (polydipsia and polyuria). * In contrast, many people with<unk>  have no signs or symptoms and are diagnosed either by accident, when a high glucose is discovered during testing for other reasons, or screening of relatives of a person discovered to have diabetes. Discovery of mild hyperglycemia during a routine glucose tolerance test for pregnancy is particularly characteristic.<unk>  cases may make up as many as 5% of presumed type 1 and type 2 diabetes cases in a large clinic population. While the goals of diabetes management are the same no matter what type, there are two primary advantages of confirming a diagnosis of<unk> . * Insulin may not be necessary and it may be possible to switch a person from insulin injections to oral agents without loss of glycemic control. * It may prompt screening of relatives and so help identify other cases in family members. As it occurs infrequently, many cases of<unk>  are initially assumed to be more common forms of diabetes: type 1 if the patient is<unk>  and not overweight, type 2 if the patient is overweight, or gestational diabetes if the patient is pregnant. Standard diabetes treatments (insulin for type 1 and gestational diabetes, and oral hypoglycemic agents for type 2) are often initiated before the doctor suspects a more unusual form of diabetes.
Maturity onset diabetes of the young
Ketosis-prone diabetes
Leucine-sensitive hypoglycemia of infancy
Cystic fibrosis–related diabetes
Lipoatrophic diabetes
Diet in diabetes
Kosaki overgrowth syndrome
Idiopathic hypoglycemia