My Approach to the Patient With Familial Hypercholesterolemia Maya S. Safarova, MD, PhD, Iftikhar J. Kullo, MD Mayo Clinic Proceedings Volume 91, Issue 6, Pages 770-786 (June 2016) DOI: 10.1016/j.mayocp.2016.04.013 Copyright © 2016 Mayo Foundation for Medical Education and Research Terms and Conditions
Figure 1 Evaluation and treatment of patients with familial hypercholesterolemia (FH). aNot at goal or intolerant. bIn patients with FH in whom maximally tolerated lipid-lowering therapy, including a statin and ezetimibe, does not lead to achievement of target low-density lipoprotein cholesterol (LDL-C) levels. cCurrently, only evolocumab, a proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitor, is approved for clinical use in homozygous FH. heFH = heterozygous FH; hoFH = homozygous FH. Mayo Clinic Proceedings 2016 91, 770-786DOI: (10.1016/j.mayocp.2016.04.013) Copyright © 2016 Mayo Foundation for Medical Education and Research Terms and Conditions
Figure 2 Low-density lipoprotein (LDL) receptor–mediated regulation of LDL cholesterol levels and drugs that affect various steps in these pathways. Apo = apolipoprotein; FFA = free fatty acids; HMG-CoA = 3-hydroxy-3-methylglutaryl coenzyme A; IDL = intermediate-density lipoprotein; MTP = microsomal triglyceride transfer protein; NPC1L1 = Niemann-Pick C1-like 1; PCSK9 = proprotein convertase subtilisin/kexin type 9; TG = triglyceride; VLDL = very low-density cholesterol. Mayo Clinic Proceedings 2016 91, 770-786DOI: (10.1016/j.mayocp.2016.04.013) Copyright © 2016 Mayo Foundation for Medical Education and Research Terms and Conditions