Sean M Peterson, Christine A Juliana, Cameron F Hu, Jinghua Chai, Carson Holliday, Kara Y Chan, Ana G Lujan Hernandez, Zoe Challocombe, Linya Wang, Zhen Han, Nikhil Haas, Ryan Stafford, Fumiko Axelrod, Tom Z Yuan, Diva D De León, Aaron K Sato
UNLABELLED: Congenital hyperinsulinism (HI) is a genetic disorder in which pancreatic β-cell insulin secretion is excessive and results in hypoglycemia that, without treatment, can cause brain damage or death. Most patients with loss-of-function mutations in ABCC8 and KCNJ11, the genes encoding the β-cell ATP-sensitive potassium channel (KATP), are unresponsive to diazoxide, the only U.S. Food and Drug Administration-approved medical therapy and require pancreatectomy. The glucagon-like peptide 1 receptor (GLP-1R) antagonist exendin-(9-39) is an effective therapeutic agent that inhibits insulin secretion in both HI and acquired hyperinsulinism...
September 1, 2023: Diabetes