LPL and acute pancreatitis: However, extremelevels of TGs are rarely explained by monogenic defects, such as familialchylomicronemia syndrome (FCS) (1).FCS is an autosomal recessive disorder that affects approximately 1 to 10individuals per million and is caused by biallelic pathogenic (P) or likelypathogenic (LP) variants in the genes encoding the lipoprotein lipase (LPL) enzyme.In addition to its heterogeneous clinical presentation, FCS is characterized by agreater risk of life-threatening, recurrent acute pancreatitis and type 3 diabetes(2).