hrp0097p1-73 | Fat, Metabolism and Obesity | ESPE2023

Insulin as a treatment modality for familial chylomicronemia syndrome in resource-limited settings- A Case series

Jebaseeli Hoole Thabitha , Suntharesan Jananie , Jayasundara Imalka , Siriwardne Dinendra , Naotunna Chamidri , Jasinghe Eresha , Atapattu Navoda

Introduction: Familial chylomicronemia syndrome (FCS) is an extremely rare monogenic disease with a prevalence of 1-2:1,000,000. Defects in lipoprotein lipase (LPL) are the main cause. Recurrent acute pancreatitis is a life-threatening complication of FCS. Insulin therapy is known to be a mode of treatment for hypertriglyceridemia.Case series: We present four children with genetically confirmed LPL deficiency followed up...