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doi:10.1016/j.pcl.2005.07.005 pediatric.theclinics.com accounts for 1% to 5% of all cases of diabetes in the United States and other industrialized countries [3]. MODY is characterized by a nonketotic hyperglycemia, pancreatic (3-cell dysfunction, and onset of disease before the age of 25 years [3]. Patients with MODY are similar to patients with type 2 diabetes mellitus in that they have a relatively mild hyperglycemia and usually do not require insulin treatment. In contrast to patients with type 2 diabetes mellitus, however, MODY patients are not obese or insulin resistant, because the primary defect in MODY is that of (-cell dysfunction. When they do require insulin, patients need very small doses (approximately 0.5 U/kg/d) to achieve good glycemic control, indicating incomplete insulin deficiency. MODY should be considered in patients who are not obese and with a two- to three-generation family history of diabetes. MODY can result from mutations in any one of six different genes including the gene encoding the glycolytic enzyme glucokinase (MODY2) and five other genes encoding transcription factors important in ( -cell development or function (Table 1).

Physiology of the fi-cell and the role of maturity-onset diabetes of the young genes

The dominant inheritance of the loss-of-function mutations that cause MODY suggests that the disease is caused by haploinsufficiency (phenotype resulting from half the normal dose) of the gene involved. This may be any of a number of genes that regulate ( -cell development, function, or both.

Table 1

Summary of MODY types and special clinical characteristics

Table 1

Summary of MODY types and special clinical characteristics

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