LPL and familial chylomicronemia syndrome: Bi-allelic pathogenic mutations in LPL, APOC2, GPIHBP1, APOA5, or LMF1, that lead to reduced LPL action, are used to confirm familial chylomicronemia syndrome, a rare autosomal recessive disorder characterized by severe elevation of TG levels, eruptive cutaneous xanthomas and acute pancreatitis (12).