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Patients with heterozygous inactivating HNF4A mutations are often born macrosomic and approximately 10% of individuals are diagnosed with hyperinsulinism within the first week of life. The clinical severity ranges from mild transient hypoglycemia that does not require pharmacological treatment to persistent HH treated with diazoxide for up to 8 years. As HNF4A mutations cause maturity-onset diabetes of the young (MODY) patients will be at increased risk of developing diabetes in later life and will often have a family history of diabetes. For further information on HNF4A-MODY see our Diabetes Genes website