[Updated on Apr. 18] Integration of CiNii Articles into CiNii Research

Guidelines for the Management of Familial Hypercholesterolemia

  • Harada-Shiba Mariko
    Department of Molecular Innovation in Lipidology, National Cerebral and Cardiovascular Center Research Institute.
  • Arai Hidenori
    Department of Human Health Sciences, Kyoto University Graduate School of Medicine
  • Oikawa Shinichi
    Division of Endocrinology and Metabolism, Department of Medicine, Nippon Medical School.
  • Ohta Takao
    Department of Child Health and Welfare (Pediatrics), Faculty of Medicine, University of the Ryukyu.
  • Okada Tomoo
    Department of Pediatrics and Child Health, Nihon University School of Medicine.
  • Okamura Tomonori
    Department of Public Health and Preventive Medicine, Keio University.
  • Nohara Atsushi
    Department of Lipidology, Kanazawa University Graduate School of Medical Science.
  • Bujo Hideaki
    Department of Genome Research and Clinical Application, Chiba University, Graduate School of Medicine.
  • Yokote Koutaro
    Department of Clinical Cell Biology and Medicine, Chiba University Graduate School of Medicine.
  • Wakatsuki Akihiko
    Department of Obstetrics and Gynecology, School of Medicine, Aichi Medical University.
  • Ishibashi Shun
    Division of Endocrinology and Metabolism, Department of Medicine, Jichi Medical University. Chair, The Research Committee for Primary Hyperlipidemia, Research on Measures against Intractable Diseases by the Japanese Ministry of Health, Labour, and Welfare.
  • Yamashita Shizuya
    Department of Cardiovascular Medicine, Osaka University Graduate School of Medicine.

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Abstract

Familial hypercholesterolemia (FH) is a highly prevalent autosomal dominant hereditary disease, generally characterized by three major signs, hyper-low-density-lipoprotein (LDL) cholesterolemia, tendon/skin xanthomas and premature coronary artery disease (CAD). Because the risk of CAD is very high in these patients, they should be identified at an early stage of their lives and started on intensive treatment to control LDL-cholesterol. We here introduce a new guideline for the management of FH patients in Japan intending to achieve better control to prevent CAD. Diagnostic criteria for heterozygous FH are 2 or more of 1) LDL-cholesterol ≥180 mg/dL, 2) tendon/skin xanthoma(s), and 3) family history of FH or premature CAD within second degree relatives, for adults; and to have both 1) LDL-cholesterol ≥140 mg/dL and 2) family history of FH or premature CAD within second degree relatives, for children. For the treatment of adult heterozygous FH, intensive lipid control with statins and other drugs is necessary. Other risks of CAD, such as smoking, diabetes mellitus, hypertension etc., should also be controlled strictly. Atherosclerosis in coronary, carotid, or peripheral arteries, the aorta and aortic valve should be screened periodically. FH in children, pregnant women, and women who wish to bear a child should be referred to specialists. For homozygotes and severe heterozygotes resistant to drug therapies, LDL apheresis should be performed. The treatment cost of homozygous FH is authorized to be covered under the program of Research on Measures against Intractable Diseases by the Japanese Ministry of Health, Labour, and Welfare.

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