Table 2: Monogenic defects associated with insulin-resistant dependent IUGR.

GeneticDefectsDiseasePathophysiology

Glucokinase (GK)Heterozigygous mutationsGlucokinase defiencyLow foetal insulin secretion
Insulin promoter factor 1 (IPF1)Homozigygous mutationPancreatic agenesisFoetal insulin secretion is abolished
Suplphonylurea-receptor1/Kir6 (SUR1/Kir6)Homozigygous mutationNesidioblastosisIncreased insulin secretion
Insulin receptor (IR)Homozigygous mutationLeprechaum syndromeMarked insulin resistance
6q22-q33Duplication or Paternal isodisomyTransient neonatal diabetesReduced insulin secretion