Case Report
Copyright ©The Author(s) 2023.
World J Diabetes. Dec 15, 2023; 14(12): 1877-1884
Published online Dec 15, 2023. doi: 10.4239/wjd.v14.i12.1877
Table 2 The clinical features of maturity-onset diabetes of the young patients
Subtype
Gene mutation
Prevalence
Clinical feature
Treatment
MODY1HNF4ACommonOne-half of patients are neonatal macrosomia; blood sugar control deteriorates gradually as the disease advances; low levels of apolipoproteins and triglycerides; without insulin resistance or β cell autoimmunityMedication-free in the early stage; sensitive to sulfonylureas
MODY2GCKCommonSlight elevation in fasting blood glucose and glycated hemoglobin levels; usually asymptomaticTypically does not require medication
MODY3HNF1ACommonRenal glucose threshold is decreased; low levels of hs-CRP; without insulin resistance or β cell autoimmunity; similar to MODY1Sensitive to sulfonylureas
MODY4PDX1/IPF1RareOverweight/obesity in some patients; commonly occurs post-puberty; postprandial blood sugar usually rises significantlyMostly treated with insulin
MODY5HNF1BUncommonOften combined with genitourinary malformations, hepatic dysfunction, renal dysfunction, renal cysts, hyperuricemia, exocrine pancreas insufficiency; onset occurs typically during adolescence or early adulthood.Early insulin therapy may be required
MODY6NEUROD1RarePhenotype is different. Overweight/obesity, intellectual disabilities and brain abnormalities occur in some patientsSignificant variations in treatment regimens
MODY7KLF11Extremely rareMild hyperglycemia, hyperlipidemiaInsulin
MODY8CELExtremely rareImpaired endocrine and exocrine pancreatic functionInsulin
MODY9PAX4Extremely rareProgressive hyperglycemia; ketoacidosis may occurMostly treated with insulin
MODY10INSRareEarlier onset of diabetes, an increased risk of diabetic microvascular complication; degree of islet dysfunction variesSignificant variations in treatment regimens
MODY11BLKExtremely rareOverweight/obesity in some patientsMost patients require insulin, but some may be treated with diet or oral hypoglycemic agents
MODY12ABCC8RareCommon in neonatal diabetes, symptoms are similar to MODY1 and 3Sensitive to sulfonylureas
MODY13KCNJ11Extremely rareCommon in neonatal diabetes, some patients develop diabetes from the second decade of life onwardsSensitive to sulfonylureas
MODY14APPL1Extremely rareOverweight/obesity in some patientsSignificant variations in treatment regimens