Maturityonset Diabetes Of The Young

Maturity-onset diabetes of the young (MODY) is an early-onset inherited form of diabetes, caused by defects in P-cell function. It was first described in a large family with autosomal dominant inheritance (1), and, subsequently, different genetically defined subgroups have been identified (2). Mildly elevated hyperglycemia may be asymptomatic and patients may be identified during intercurrent illness or on screening during pregnancy (3). The criteria for diagnosis (2) are as follows:

• Early-onset non-insulin-dependent diabetes. At least one family member diagnosed under 25 yr; non-insulin dependent if 5 yr after diagnosis the person is not on insulin or, if treated with insulin, has significant circulating C-peptide.

• Autosomal dominant inheritance. At least two and, ideally, three generations are affected with diabetes.

Maturity-onset diabetes of the young is thought to account for 1-2% of patients in adult type 2 diabetes clinics. The proportion in pediatric diabetes clinics is unknown, but it is probably much more common than the other single-gene defects described in this chapter. In a young person presenting with diabetes, the differential is usually

Table 2

Comparison of MODY, Type 1 Diabetes, and Type 2 Diabetes

Table 2

Comparison of MODY, Type 1 Diabetes, and Type 2 Diabetes

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