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©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
Published online Dec 15, 2023. doi: 10.4239/wjd.v14.i12.1877
Subtype | Gene mutation | Prevalence | Clinical feature | Treatment |
MODY1 | HNF4A | Common | One-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 autoimmunity | Medication-free in the early stage; sensitive to sulfonylureas |
MODY2 | GCK | Common | Slight elevation in fasting blood glucose and glycated hemoglobin levels; usually asymptomatic | Typically does not require medication |
MODY3 | HNF1A | Common | Renal glucose threshold is decreased; low levels of hs-CRP; without insulin resistance or β cell autoimmunity; similar to MODY1 | Sensitive to sulfonylureas |
MODY4 | PDX1/IPF1 | Rare | Overweight/obesity in some patients; commonly occurs post-puberty; postprandial blood sugar usually rises significantly | Mostly treated with insulin |
MODY5 | HNF1B | Uncommon | Often 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 |
MODY6 | NEUROD1 | Rare | Phenotype is different. Overweight/obesity, intellectual disabilities and brain abnormalities occur in some patients | Significant variations in treatment regimens |
MODY7 | KLF11 | Extremely rare | Mild hyperglycemia, hyperlipidemia | Insulin |
MODY8 | CEL | Extremely rare | Impaired endocrine and exocrine pancreatic function | Insulin |
MODY9 | PAX4 | Extremely rare | Progressive hyperglycemia; ketoacidosis may occur | Mostly treated with insulin |
MODY10 | INS | Rare | Earlier onset of diabetes, an increased risk of diabetic microvascular complication; degree of islet dysfunction varies | Significant variations in treatment regimens |
MODY11 | BLK | Extremely rare | Overweight/obesity in some patients | Most patients require insulin, but some may be treated with diet or oral hypoglycemic agents |
MODY12 | ABCC8 | Rare | Common in neonatal diabetes, symptoms are similar to MODY1 and 3 | Sensitive to sulfonylureas |
MODY13 | KCNJ11 | Extremely rare | Common in neonatal diabetes, some patients develop diabetes from the second decade of life onwards | Sensitive to sulfonylureas |
MODY14 | APPL1 | Extremely rare | Overweight/obesity in some patients | Significant variations in treatment regimens |
- Citation: Chen H, Fei SJ, Deng MQ, Chen XD, Wang WH, Guo LX, Pan Q. Maturity-onset diabetes of the young type 10 caused by an Ala2Thr mutation of INS: A case report. World J Diabetes 2023; 14(12): 1877-1884
- URL: https://www.wjgnet.com/1948-9358/full/v14/i12/1877.htm
- DOI: https://dx.doi.org/10.4239/wjd.v14.i12.1877