血管性水肿

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血管神经性水肿
同義詞 血管性水肿,Angiooedema, Quincke's edema, angioneurotic edema
Angioedema2010.JPG
血管神经性水肿: 患儿由于眼皮肿胀不能睁眼.
醫學專科 免疫学
症状 局部肿胀 [1]
常見始發於 几分钟到几小时[1]
種類 组胺调节, 缓激肽调节[1]
風險因子英语Risk factor 家族史[2]
診斷方法 对症治疗[2]
相似疾病或共病 全身型過敏性反應, 膿瘍, 接触性皮炎英语contact dermatitis[2]
治療 插管, 环甲状软骨切开术英语cricothyroidotomy[1]
藥物 组胺: 抗組織胺藥, 皮質類固醇, 肾上腺素[1]
缓激肽: C1酯酶抑制物英语C1 esterase inhibitor, 艾卡拉肽英语ecallantide, 艾替班特英语icatibant, 新鲜冷冻血浆英语fresh frozen plasma[1]
盛行率 ~100,000每年(美国)[1]

血管神经性水肿真皮皮下组织英语subcutaneous tissue黏膜的局部肿胀。[1][3]可发生于面部、舌头、、腹部、四肢。[1]常与荨麻疹相关,荨麻疹是皮肤的红肿。[1][3] Onset is typically over minutes to hours.[1]

基本机制涉及组胺缓激肽[1] 与组胺相关的是由于对过敏原的过敏反应,如蚊虫叮咬,食物,或药品[1] 与缓激肽相关的是遗传问题称作获得性C1酯酶抑制剂缺乏英语C1 esterase inhibitor deficiency,药物有血管紧张素转换酶抑制剂, 或淋巴组织增生性疾病英语lymphoproliferative disorder.[1]

为保护呼吸道通畅,对呼吸道特别是喉部发作水肿,必要时应进行气管插管环甲状软骨切开术英语cricothyroidotomy[1]组胺相关血管神经性水肿可抗組織胺藥:对症治疗常采用抗组胺受体H1拮抗剂,对顽固的、应用抗组胺受体拮抗剂无效的患者,可合并应用抗组胺受体H2拮抗剂如西咪替丁(甲氰咪呱)或兰替丁,有时可取得满意效果。酮体芬亦可合并使用。拟交感神经药物主要用于急性荨麻疹和(或)神经性水肿,尤其是喉水肿患者,应用0.1%肾上腺素皮下注射,对严重急性过敏性反应可隔20~30分钟注射。同时给予糖皮質類固醇激素静脉滴注,氨茶碱口服或静脉注射。[1] 缓激肽相关的疾病可用C1酯酶抑制物英语C1 esterase inhibitor, 艾卡拉肽英语ecallantide, 艾替班特英语icatibant治疗。[1] Fresh frozen plasma may be used instead.[1] In the United States the disease affects about 100,000 people a year.[1]

病理学[编辑]

缓激肽在各种血管性水肿中扮演了关键角色。[9] 这种是强力血管舒張并增加血管通透,导致组织液快速累积。这在脸部特别显著,因为脸部皮肤相对缺少结缔组织支撑,容易形成水肿。多种细胞类型在刺激下会释放缓激肽。不同的干涉缓激肽生产或讲解的机制能导致血管性水肿。ACE inhibitors block 血管紧张素转化酶(ACE)能降解缓激肽。遗传性血管性水肿英语hereditary angioedema是导致缓激肽生成的补体系统持续活化,因为缺乏相应的抑制剂:C1酯酶(C1INH)。这种丝氨酸蛋白酶抑制剂能抑制C1r、C1s与C1q的关联,阻止了C1复合体的生成,从而激活了其他的补体系统的蛋白。此外还抑制了相应的一些凝血蛋白,虽然对出血血栓形成的干扰效果是有限的。

有三类遗传性血管性水肿英语hereditary angioedema

血管性水肿也可能是生成了C1INH抗体,这是一种自體免疫性疾病。与淋巴瘤的发展有关。

食物消化吸收后可能带来自己的血管扩张剂,如酒精饮料桂皮,可加重病情。菠萝蛋白酶薑黃合用能减轻症状。[12]中药分别称两种效果为“发货”与抗炎消肿。

布洛芬阿司匹林可能会加重病情。对乙酰氨基酚也能加重病情。

获得性[编辑]

获得性血管神经性水肿、HAE的I型与II型、以及非组胺血管神经性水肿性,抗纤维蛋白溶酶药物如氨甲环酸或ε-氨基己酸有时可控制自然发作,也适用于小儿和孕妇。桂利嗪英语Cinnarizine可用于抗C4活化,适用于有肝病不能使用雄性激素的患者。[22]

历史[编辑]

1882年,Heinrich Quincke英语Heinrich Quincke首次临床报告此病。[23] 虽然有些更早的临床描述。[24][25][26]

1888年,William Osler英语William Osler认为某些是由于遗传导致的,称“遗传性血管神经性水肿”。[27]

1963年证实了C1酯酶抑制剂匮乏是病因。[28]

流行病学[编辑]

在美国,急诊室年度就诊患者中有80,000至112,000是血管神经性水肿,是过敏导致就医中的首位。[29]

参见[编辑]

参考文献[编辑]

  1. ^ 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 1.11 1.12 1.13 1.14 1.15 1.16 1.17 1.18 1.19 Bernstein, JA; Cremonesi, P; Hoffmann, TK; Hollingsworth, J. Angioedema in the emergency department: a practical guide to differential diagnosis and management.. International journal of emergency medicine. December 2017, 10 (1): 15. PMID 28405953. 
  2. ^ 2.0 2.1 2.2 Caterino, Jeffrey M.; Kahan, Scott. In a Page: Emergency medicine. Lippincott Williams & Wilkins. 2003: 133. ISBN 9781405103572. (原始内容存档于2017-09-10) (英语). 
  3. ^ 3.0 3.1 Habif, Thomas P. Clinical Dermatology E-Book 5. Elsevier Health Sciences. 2009: 182. ISBN 0323080375. (原始内容存档于2017-09-10) (英语). 
  4. ^ Bork K; Barnstedt Se. Laryngeal edema and death from asphyxiation after tooth extraction in four patients with hereditary angioedema. J Am Dent Assoc. August 2003, 134 (8): 1088–94. PMID 12956349. doi:10.14219/jada.archive.2003.0323. (原始内容存档于2012-07-23). 
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  8. ^ Loew, Burr. A 68-Year-Old Woman With Recurrent Abdominal Pain, Nausea, and Vomiting. MedScape. [19 October 2012]. (原始内容存档于22 October 2012). 
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  11. ^ Cichon S, Martin L, Hennies HC, 等. Increased activity of coagulation factor XII (Hageman factor) causes hereditary angioedema type III. Am. J. Hum. Genet. 2006, 79 (6): 1098–104. PMC 1698720. PMID 17186468. doi:10.1086/509899. 
  12. ^ University of Maryland Medical Center. Angioedema. Archived copy. [2008-01-08]. (原始内容存档于2007-10-12). 
  13. ^ 13.0 13.1 Sabroe RA, Black AK. Angiotensin-converting enzyme (ACE) inhibitors and angio-oedema. British Journal of Dermatology. February 1997, 136 (2): 153–8. PMID 9068723. doi:10.1111/j.1365-2133.1997.tb14887.x. 
  14. ^ 14.0 14.1 Israili ZH, Hall WD. Cough and angioneurotic edema associated with angiotensin-converting enzyme inhibitor therapy. A review of the literature and pathophysiology. Annals of Internal Medicine. August 1, 1992, 117 (3): 234–42. PMID 1616218. doi:10.7326/0003-4819-117-3-234. 
  15. ^ Kostis JB, Kim HJ, Rusnak J, Casale T, Kaplan A, Corren J, Levy E. Incidence and characteristics of angioedema associated with enalapril. Archives of Internal Medicine. July 25, 2005, 165 (14): 1637–42. PMID 16043683. doi:10.1001/archinte.165.14.1637. 
  16. ^ Brown NJ, Ray WA, Snowden M, Griffin MR. Black Americans have an increased rate of angiotensin converting enzyme inhibitor-associated angioedema. Clinical Pharmacologic Therapy. July 1996, 60 (1): 8–13. PMID 8689816. doi:10.1016/S0009-9236(96)90161-7. 
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  19. ^ Cicardi M, Zingale LC, Bergamaschini L, Agostoni A. Angioedema associated with angiotensin-converting enzyme inhibitor use: outcome after switching to a different treatment. Archives of Internal Medicine. April 26, 2004, 164 (8): 910–3. PMID 15111379. doi:10.1001/archinte.164.8.910. 
  20. ^ LLC, Prime Therapeutics. Study: Drug Costs for Rare Hereditary Angioedema Disorder Tripled in Two Years. www.prnewswire.com. (原始内容存档于2015-10-25) (英语). 
  21. ^ Drouet C, Désormeaux A, Robillard J, Ponard D, Bouillet L, Martin L, 等. Metallopeptidase activities in hereditary angioedema: effect of androgen prophylaxis on plasma aminopeptidase P. The Journal of Allergy and Clinical Immunology. 2008, 121 (2): 429–33. PMID 18158172. doi:10.1016/j.jaci.2007.10.048. 
  22. ^ Archived copy (PDF). [2007-01-26]. (原始内容 (PDF)存档于2007-09-28). 
  23. ^ Quincke H. Über akutes umschriebenes Hautödem. Monatsh Prakt Derm. 1882, 1: 129–131. 
  24. ^ synd/482 - Who Named It?英语Who Named It?
  25. ^ Marcello Donati. De medica historia mirabili. Mantuae, per Fr. Osanam, 1586
  26. ^ J. L. Milton. On giant urticaria. Edinburgh Medical Journal, 1876, 22: 513-526.
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  28. ^ Donaldson VH, Evans RR. A biochemical abnormality in hereditary angioneurotic edema: absence of serum inhibitor of C' 1-esterase. Am. J. Med. July 1963, 35: 37–44. PMID 14046003. doi:10.1016/0002-9343(63)90162-1. 
  29. ^ http://healthnews.uc.edu/news/?/24791/ 互联网档案馆存檔,存档日期2014-07-14.

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