지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2021.1
- 수록면
- 279 - 295 (17page)
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초록· 키워드
오류제보하기
Patients with familial hypercholesterolemia (FH) are at high or very high risk for cardiovascular disease. Those with heterozygousFH (HeFH) often do not reach low-density lipoprotein cholesterol (LDL-C) targets with statin and ezetimibe therapy, and those withhomozygous FH (HoFH) usually require additional lipid-modifying therapies. Drugs that inhibit proprotein convertase subtilisin/kexin type 9 (PCSK9) offer a novel approach to reduce LDL-C. The monoclonal antibodies, alirocumab and evolocumab, given bysubcutaneous injection every 2 or 4 weeks produce reductions in LDL-C of 50% to 60% in patients with HeFH, allowing many ofthem to achieve their LDL-C goals. Patients with HoFH show a reduced and more variable LDL-C response, which appears to depend on residual LDL receptor activity, and those with receptor-negative mutations may show no response. Inclisiran is a long-actingsmall interfering RNA therapeutic agent that inhibits the synthesis of PCSK9. Subcutaneous doses of 300 mg can reduce LDL-C bymore than 50% for at least 6 months and the responses in HeFH and HoFH patients are similar to those achieved with monoclonalantibodies. These PCSK9 inhibitors are generally well tolerated and they provide a new opportunity for effective treatment for themajority of patients with FH.
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참고문헌 (108)
참고문헌 신청
Nordestgaard BG / 2013 / Familial hypercholesterolaemia is underdiagnosed and undertreated in the gen-eral population : guidance for clinicians to prevent coronary heart disease : consensus statement of the European Atherosclerosis Society / Eur Heart J 34:3478~3490
Akioyamen LE / 2017 / Estimating the prevalence of heterozygous familial hypercholesterolaemia : a systematic review and meta-analysis / BMJ Open 7:e016461
Sturm AC / 2018 / Clinical genetic testing for familial hypercholesterolemia : JACC Scientific Expert Panel / J Am Coll Cardiol 72:662~680
Foody JM / 2016 / Familial hypercholesterolemia/autosomal dominant hypercholesterolemia : molecular defects, the LDL-C continuum, and gradients of phenotypic severity / J Clin Lipidol 10:970~986
Cuchel M / 2014 / Homozygous familial hypercholesterolaemia : new insights and guidance for clinicians to improve detection and clinical management : a position paper from the Consensus Panel on Familial Hypercholesterolaemia of the European Atherosclerosis Society / Eur Heart J 35:2146~2157
Akioyamen LE / 2017 / Estimating the prevalence of heterozygous familial hypercholesterolaemia : a systematic review and meta-analysis / BMJ Open 7:e016461
Sturm AC / 2018 / Clinical genetic testing for familial hypercholesterolemia : JACC Scientific Expert Panel / J Am Coll Cardiol 72:662~680
Foody JM / 2016 / Familial hypercholesterolemia/autosomal dominant hypercholesterolemia : molecular defects, the LDL-C continuum, and gradients of phenotypic severity / J Clin Lipidol 10:970~986
Cuchel M / 2014 / Homozygous familial hypercholesterolaemia : new insights and guidance for clinicians to improve detection and clinical management : a position paper from the Consensus Panel on Familial Hypercholesterolaemia of the European Atherosclerosis Society / Eur Heart J 35:2146~2157
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