Copyright ©The Author(s) 2024.
World J Diabetes. Jan 15, 2024; 15(1): 15-23
Published online Jan 15, 2024. doi: 10.4239/wjd.v15.i1.15
Table 1 Classification of maturity-onset diabetes of the young
MODY type
MODY sub-type
Genes affected
Characteristics features
HNF4A-MODYMODY 1HNF4AYoung-onset hyperglycemia, pancreatic beta cell dysfunction, sensitive to sulfonylurea treatment, macrosomia, transient neonatal hyperinsulinism
GCK-MODYMODY 2GCKMild fasting hyperglycemia, stable glucose levels, lack of complications
HNF1A-MODYMODY 3TCF1Young-onset hyperglycemia, sensitive to sulfonylurea treatment, renal cysts, genital tract anamolies
PDX1-MODYMODY 4PDX1Young-onset hyperglycemia, pancreatic agenesis, hypopituitarism, growth retardation
HNF1B-MODYMODY 5HNF1BYoung-onset hyperglycemia, renal abnormalities, genital tract malformations, gout
NEUROD1-MODYMODY 6NEUROD1Young-onset hyperglycemia, retinal dystrophy, cerebellar ataxia, epilepsy, intellectual disability, sensorineural hearing loss
KLF11-MODYMODY 7KLF11Young-onset hyperglycemia, hepatic steatosis
CEL-MODYMODY 8CELNeonatal diabetes, pancreatic atrophy, exocrine pancreatic insufficiency, transient neonatal hyperinsulinism
PAX4-MODYMODY 9PAX4Adult onset diabetes, multisystem disorder, mutation inhibits beta-cell proliferation, ketosis prone
INS-MODYMODY 10INSNeonatal diabetes, insulin gene mutation, requires lifelong insulin treatment
BLK-MODYMODY 11BLKYoung-onset hyperglycemia, reduced beta-cell mass
ABCC8-MODYMODY 12ABCC8Neonatal diabetes, potassium channel gene mutation, responsive to high-dose sulfonylurea
KCNJ11-MODYMODY 13KCNJ11Neonatal diabetes, potassium channel gene mutation, responsive to high-dose sulfonylurea
APPL1-MODYMODY 14APPL1Young-onset diabetes and decreased glucose mediated insulin release, dysmorphic features and developmental delay in animal models
Table 2 Complications of glucokinase maturity-onset diabetes of the young in pregnancy
Outcome feature
Preterm birth12%[27]
Low birth weight6%[28]
Neonatal hypoglycemia10%[23]
Congenital malformations2%-3%[23]