Hypoglycemia and antihyperglycemic treatment in adult MODY patients - A systematic review of literature.

Maturity onset diabetes of the young (MODY) is a heterogeneous group of diseases caused by a single mutation in one of the 14 genes involved in the regulation of glucose homeostasis. GCK, HNF1A, and HNF4A genes are among the most common genes affected. Expression of these genes in the key organs for defense against hypoglycemia and their participation in counter-regulation to hypoglycemia may potentially put individuals with a heterozygous mutation in these genes at increased risk for hypoglycemia. In HNF4A-MODY and HNF1A-MODY patients, normal or even increased insulin sensitivity together with glucose-independent mechanism of action of the first-line therapy - sulphonylurea derivatives - often leads to hypoglycemia, even at the much lower dose used in type 2 diabetes. This review aims to analyze clinical studies and case reports concerning hypoglycemia associated with antihyperglycemic treatment in adult MODY patients.