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遗传性血管性水肿治疗进展:皮下注射 C1 抑制剂替代疗法预防血管性水肿发作

Advances in Hereditary Angioedema: The Prevention of Angioedema Attacks With Subcutaneous C1-Inhibitor Replacement Therapy.

机构信息

AARA Research Center, Dallas, Texas (Dr Lumry); US HAEA Angioedema Center at University of California San Diego, San Diego, California (Ms Templeton); CSL Behring, King of Prussia, Pennsylvania (Dr Omert); University of California Irvine School of Medicine, Orange, California (Dr Levy).

William Lumry, MD, is in private practice in Dallas, Texas, treating adults and children with allergic diseases and asthma. He actively participates in clinical research projects involving new treatments for asthma, allergic and immune deficiency diseases, and hereditary angioedema in his role as medical director of AARA Research Center. He is also clinical professor of internal medicine at the University of Texas Southwestern Medical School in Dallas and teaches at Parkland Memorial Hospital in Dallas. Dr Lumry is president of the Texas Allergy, Asthma and Immunology Society and a fellow of the American Academy of Allergy, Asthma and Immunology, the American College of Allergy, Asthma and Immunology, and the American College of Physicians. He is also a member of the US Hereditary Angioedema Association Medical Advisory Board on the panels responsible for the Hereditary Angioedema International Working Group, International Consensus, and World Allergy Organization hereditary angioedema consensus documents.

出版信息

J Infus Nurs. 2020 May/Jun;43(3):134-145. doi: 10.1097/NAN.0000000000000365.

DOI:10.1097/NAN.0000000000000365
PMID:32287168
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7328861/
Abstract

Hereditary angioedema (HAE) is a debilitating condition caused by a functional C1-inhibitor (C1-INH) deficiency and characterized clinically by episodes of subcutaneous or submucosal swelling. C1-INH replacement is highly effective for preventing HAE attacks and can improve health-related quality of life. Once available only for intravenous use, C1-INH is now available as a subcutaneous formulation for self-administration, shown to provide sustained plasma levels of C1-INH and reducing the monthly median HAE attack rate by 95% versus placebo in the phase 3 COMPACT study. Subcutaneously administered C1-INH satisfies multiple unmet needs in the management of patients with HAE.

摘要

遗传性血管性水肿(HAE)是一种由功能性 C1 抑制剂(C1-INH)缺乏引起的使人虚弱的疾病,临床上表现为皮下或黏膜下肿胀发作。C1-INH 替代疗法对预防 HAE 发作非常有效,并能改善与健康相关的生活质量。C1-INH 曾经仅可用于静脉使用,现在已有皮下制剂供自我给药,在 3 期 COMPACT 研究中,与安慰剂相比,它显示出能持续提供 C1-INH 的血浆水平,并将每月 HAE 发作的中位数降低 95%。皮下给予 C1-INH 满足了 HAE 患者管理中的多种未满足需求。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/69a8/7328861/b9f845ddddd8/jinfn-43-134-g006.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/69a8/7328861/d5a75a6ca594/jinfn-43-134-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/69a8/7328861/8b61e627718a/jinfn-43-134-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/69a8/7328861/2079fe043da2/jinfn-43-134-g003.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/69a8/7328861/b9f845ddddd8/jinfn-43-134-g006.jpg

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本文引用的文献

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J Allergy Clin Immunol Pract. 2019 Jul-Aug;7(6):1793-1802.e2. doi: 10.1016/j.jaip.2019.01.054. Epub 2019 Feb 15.
2
Training patients for self-administration of a new subcutaneous C1-inhibitor concentrate for hereditary angioedema.对患者进行培训,使其能够自行注射一种用于遗传性血管性水肿的新型皮下注射C1抑制剂浓缩物。
Nurs Open. 2018 Aug 28;6(1):126-135. doi: 10.1002/nop2.194. eCollection 2019 Jan.
3
Effect of Lanadelumab Compared With Placebo on Prevention of Hereditary Angioedema Attacks: A Randomized Clinical Trial.
拉那芦单抗对比安慰剂预防遗传性血管性水肿发作的效果:一项随机临床试验。
JAMA. 2018 Nov 27;320(20):2108-2121. doi: 10.1001/jama.2018.16773.
4
Treatment patterns and healthcare resource utilization among patients with hereditary angioedema in the United States.美国遗传性血管性水肿患者的治疗模式和医疗资源利用情况。
Orphanet J Rare Dis. 2018 Oct 12;13(1):180. doi: 10.1186/s13023-018-0922-3.
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Subcutaneous C1-esterase inhibitor to prevent hereditary angioedema attacks: Safety findings from the COMPACT trial.皮下注射C1酯酶抑制剂预防遗传性血管性水肿发作:COMPACT试验的安全性结果
Allergy Asthma Proc. 2018 Sep 14;39(5):365-370. doi: 10.2500/aap.2018.39.4164. Epub 2018 Aug 14.
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ACE Inhibitor-Induced Angioedema: a Review.血管紧张素转换酶抑制剂相关性血管水肿:综述。
Curr Hypertens Rep. 2018 Jun 8;20(7):55. doi: 10.1007/s11906-018-0859-x.
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Epidemiology of Bradykinin-mediated angioedema: a systematic investigation of epidemiological studies.缓激肽介导的血管性水肿的流行病学:对流行病学研究的系统调查。
Orphanet J Rare Dis. 2018 May 4;13(1):73. doi: 10.1186/s13023-018-0815-5.
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Hereditary angioedema with normal C1 inhibitor: Four types and counting.C1抑制剂正常的遗传性血管性水肿:已有四种类型且仍在增加。
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Health-Related Quality of Life with Subcutaneous C1-Inhibitor for Prevention of Attacks of Hereditary Angioedema.用于预防遗传性血管性水肿发作的皮下 C1 抑制剂的健康相关生活质量。
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