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1
Therapy in hereditary angioneurotic oedema.遗传性血管性水肿的治疗
Arch Dis Child. 1974 Aug;49(8):636-40. doi: 10.1136/adc.49.8.636.
2
Hereditary angioneurotic oedema in three families. Symptomatic heterogeneity, complement analysis and therapeutic trials.三个家族中的遗传性血管性水肿。症状异质性、补体分析及治疗试验。
Acta Med Scand. 1972 Mar;191(3):209-19.
3
Hereditary angio-neurotic oedema. Genealogical and immunological studies.遗传性血管神经性水肿。系谱学和免疫学研究。
Ann Clin Res. 1973 Jun;5(3):174-80.
4
Epsilon aminocaproic acid therapy of hereditary angioneurotic edema. A double-blind study.遗传性血管性水肿的ε-氨基己酸治疗。一项双盲研究。
N Engl J Med. 1972 Apr 13;286(15):808-12. doi: 10.1056/NEJM197204132861503.
5
A family study of hereditary angioneurotic oedema.遗传性血管性水肿的家系研究
Q J Med. 1973 Apr;42(166):317-39.
6
Hereditary angio-oedema treated with E-aminocaproic acid.用ε-氨基己酸治疗遗传性血管性水肿。
Br J Dermatol. 1969 Oct;81(10):763-5. doi: 10.1111/j.1365-2133.1969.tb15938.x.
7
A case of hereditary angioneurotic oedema, successfully treated with epsilon-aminocaproic acid. Studies on C'1 esterase inhibitor, C'1 activation, plasminogen level and histamine metabolism.1例遗传性血管神经性水肿，用6-氨基己酸成功治疗。关于C1酯酶抑制剂、C1激活、纤溶酶原水平及组胺代谢的研究。
Clin Exp Immunol. 1968 Sep;3(7):733-45.
8
Prophylactic use of epsilon aminocaproic acid for oral surgery in a patient with hereditary angioneurotic edema.遗传性血管性水肿患者口腔手术中ε-氨基己酸的预防性应用。
J Allergy Clin Immunol. 1974 May;53(5):298-302. doi: 10.1016/0091-6749(74)90109-2.
9
[A molecular disease: hereditary angioneurotic edema. Study of 16 families].[一种分子疾病：遗传性血管性水肿。对16个家族的研究]
Biomedicine. 1974 Jul;21(7):294-302.
10
Angioneurotic edema. A review of modern concepts.血管性水肿。现代概念综述。
Arch Otolaryngol. 1975 Sep;101(9):544-7. doi: 10.1001/archotol.1975.00780380022005.

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The international WAO/EAACI guideline for the management of hereditary angioedema - The 2021 revision and update.国际血管性水肿学会（WAO）/欧洲变态反应和临床免疫学会（EAACI）遗传性血管性水肿管理指南——2021年修订与更新
World Allergy Organ J. 2022 Apr 7;15(3):100627. doi: 10.1016/j.waojou.2022.100627. eCollection 2022 Mar.
2
The International/Canadian Hereditary Angioedema Guideline.《国际/加拿大遗传性血管性水肿指南》
Allergy Asthma Clin Immunol. 2019 Nov 25;15:72. doi: 10.1186/s13223-019-0376-8. eCollection 2019.
3
Canadian hereditary angioedema guideline.加拿大遗传性血管性水肿指南。
Allergy Asthma Clin Immunol. 2014 Oct 24;10(1):50. doi: 10.1186/1710-1492-10-50. eCollection 2014.
4
Hereditary angioedema (HAE) in children and adolescents--a consensus on therapeutic strategies.儿童和青少年遗传性血管性水肿（HAE）——治疗策略的共识。
Eur J Pediatr. 2012 Sep;171(9):1339-48. doi: 10.1007/s00431-012-1726-4. Epub 2012 Apr 29.
5
Management of upper airway edema caused by hereditary angioedema.遗传性血管性水肿所致上呼吸道水肿的管理。
Allergy Asthma Clin Immunol. 2010 Jul 28;6(1):19. doi: 10.1186/1710-1492-6-19.
6
C1-inhibitor concentrate home therapy for hereditary angioedema: a viable, effective treatment option.遗传性血管性水肿的C1抑制剂浓缩物家庭治疗：一种可行、有效的治疗选择。
Clin Exp Immunol. 2007 Jan;147(1):11-7. doi: 10.1111/j.1365-2249.2006.03256.x.
7
C1 inhibitor deficiency: consensus document.C1 抑制剂缺乏症：共识文件。
Clin Exp Immunol. 2005 Mar;139(3):379-94. doi: 10.1111/j.1365-2249.2005.02726.x.
8
Current management of hereditary angio-oedema (C'1 esterase inhibitor deficiency).遗传性血管性水肿（C1酯酶抑制剂缺乏症）的当前管理
J Clin Pathol. 2002 Apr;55(4):266-70. doi: 10.1136/jcp.55.4.266.

本文引用的文献

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Hereditary angioneurotic edema. I. Case reports and review of the literature.遗传性血管性水肿。I. 病例报告及文献综述
J Allergy. 1962 Jul-Aug;33:316-29. doi: 10.1016/0021-8707(62)90031-x.
2
EPSILON-AMINOCAPROIC ACID AND OTHER INHIBITORS OF FIBRINOLYSIS.ε-氨基己酸及其他纤维蛋白溶解抑制剂
Br Med Bull. 1964 Sep;20:233-9. doi: 10.1093/oxfordjournals.bmb.a070338.
3
The inactivation of complement and its components by plasmin.纤溶酶对补体及其成分的灭活作用。
J Exp Med. 1953 Apr;97(4):573-89. doi: 10.1084/jem.97.4.573.
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Hereditary angioneurotic edema: a clinical survey.遗传性血管性水肿：一项临床调查。
Pediatrics. 1966 Jun;37(6):1017-27.
5
Immunochemical quantitation of antigens by single radial immunodiffusion.通过单向辐射免疫扩散法对抗原进行免疫化学定量。
Immunochemistry. 1965 Sep;2(3):235-54. doi: 10.1016/0019-2791(65)90004-2.
6
Hereditary angio-oedema treated with E-aminocaproic acid.用ε-氨基己酸治疗遗传性血管性水肿。
Br J Dermatol. 1969 Oct;81(10):763-5. doi: 10.1111/j.1365-2133.1969.tb15938.x.
7
Tranexamic acid therapy in hereditary angioneurotic edema.氨甲环酸治疗遗传性血管性水肿。
N Engl J Med. 1972 Aug 31;287(9):452-4. doi: 10.1056/NEJM197208312870907.
8
Epsilon aminocaproic acid therapy of hereditary angioneurotic edema. A double-blind study.遗传性血管性水肿的ε-氨基己酸治疗。一项双盲研究。
N Engl J Med. 1972 Apr 13;286(15):808-12. doi: 10.1056/NEJM197204132861503.
9
Hereditary angio-oedema: a review with particular reference to pathogenesis and treatment.遗传性血管性水肿：特别涉及发病机制与治疗的综述
Clin Allergy. 1971 Jun;1(2):221-33. doi: 10.1111/j.1365-2222.1971.tb03021.x.
10
Replacement therapy in hereditary angioedema. Successful treatment of two patients with fresh frozen plasma.遗传性血管性水肿的替代疗法。两例新鲜冷冻血浆治疗成功病例
Lancet. 1969 Feb 15;1(7590):326-30. doi: 10.1016/s0140-6736(69)91295-1.

遗传性血管性水肿的治疗

Therapy in hereditary angioneurotic oedema.

作者信息

Gwynn C M

出版信息

Arch Dis Child. 1974 Aug;49(8):636-40. doi: 10.1136/adc.49.8.636.

DOI:10.1136/adc.49.8.636
PMID:4213636
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1648996/
Abstract

Two branches of a family suffering from hereditary angioneurotic oedema underwent trials of therapy of ε-amino caproic-acid (EACA) to ascertain the optimum dosage required to alleviate symptoms without giving rise to unpleasant side effects. It was found that children under 11 years tolerated 3 g/day and patients over 11 years 6 g/day without side effects, but with incomplete control of symptoms in some of the patients. However, if the dose was doubled for a period of less than 4 days during times of expected and experienced trauma, better control was achieved and unpleasant muscle cramps were not experienced. These doses, though effective, were much smaller than those used in previous studies.

摘要

两个患有遗传性血管性水肿的家族分支接受了ε-氨基己酸（EACA）治疗试验，以确定缓解症状且不产生不良副作用所需的最佳剂量。结果发现，11岁以下儿童耐受3克/天，11岁以上患者耐受6克/天，均无副作用，但部分患者症状控制不完全。然而，如果在预期和经历创伤期间将剂量加倍不到4天，则可实现更好的控制，且未出现令人不适的肌肉痉挛。这些剂量虽然有效，但比以前研究中使用的剂量小得多。