hrp0097p1-73 | Fat, Metabolism and Obesity | ESPE2023
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...