LPL and familial chylomicronemia syndrome: Rarely, patients with sHTG have chylomicronemia that is monogenic in origin, arising due to loss-of-function (LOF) mutations in genes encoding LPL or other genes of the LPL pathway, including the genes encoding apolipoprotein (APO) A5 (APOA5), APOC2 (APOC2), glycosylphosphatidylinositol anchored high-density lipoprotein binding protein 1 (GPIHBP1) and lipase maturation factor 1 (LMF1), resulting in familial chylomicronemia syndrome (FCS)7,9.