Case Report: long-term misdiagnosis and follow-up of a patient with HNF4A-MODY carrying a new de novo mutation

This case emphasizes that spontaneous <i>HNF4A</i>-MODY is prone to misdiagnosis and the prolonged rate of pancreatic function decline in <i>HNF4A</i>-MODY. Glycemic control and complication progression could be acceptable in <i>HNF4A</i>-MODY cases treated with long-time insulin, but risks of hypoglycemic events, obesity, and atherosclerosis remain. Switching to GLP1RA treatment in <i>HNF4A</i>-MODY still yields a good effect after a prolonged disease course.