血管性水肿
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血管神经性水肿(英语:Angioedema)或血管性水肿是真皮、皮下组织、黏膜的局部肿胀。[1][3]可发生于面部、舌头、喉、腹部、四肢。[1]常与荨麻疹相关,荨麻疹是皮肤的红肿。[1][3]约数分钟至数小时内发病。[1]
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血管神经性水肿 | |
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同义词 | 血管性水肿,Angiooedema, Quincke's edema, angioneurotic edema |
血管神经性水肿: 患儿由于眼皮肿胀不能睁眼 | |
症状 | 局部肿胀[1] |
起病年龄 | 几分钟到几小时[1] |
类型 | 组胺调节, 缓激肽调节[1] |
风险因素 | 家族史[2] |
诊断方法 | 对症治疗[2] |
鉴别诊断 | 全身型过敏性反应, 脓疡, 接触性皮炎[2] |
治疗 | 插管, 环甲状软骨切开术(英语:cricothyroidotomy)[1] |
药物 | 组胺: 抗组织胺药, 皮质类固醇, 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[1] |
患病率 | ~100,000每年(美国)[1] |
分类和外部资源 | |
医学专科 | 免疫学 |
ICD-9-CM | 995.1 |
OMIM | 106100、610618、106100、610618 |
DiseasesDB | 13606 |
MedlinePlus | 000846 |
eMedicine | 756261、135208、885100 |
[编辑此条目的维基数据] |
基本机制涉及组胺或缓激肽。[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或环甲膜切开术。[1]组胺相关血管神经性水肿可抗组织胺药: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。同时给予糖皮质类固醇激素静脉滴注,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] 新鲜冷冻血浆也可作为替代疗法。[1]美国每年约十万人发生此病。[1]
症状
![](http://upload.wikimedia.org/wikipedia/commons/thumb/7/7d/Blausen_0023_Angioedema.png/640px-Blausen_0023_Angioedema.png)
The skin of the face, normally around the mouth, and the mucosa of the mouth and/or throat, as well as the tongue, swell over the period of minutes to hours. The swelling can also occur elsewhere, typically in the hands. The swelling can be itchy or painful. There may also be slightly decreased sensation in the affected areas due to compression of the nerves. Urticaria (hives) may develop simultaneously.
In severe cases, stridor of the airway occurs, with gasping or wheezy inspiratory breath sounds and decreasing oxygen levels. Tracheal intubation is required in these situations to prevent respiratory arrest and risk of death.
Sometimes, the cause is recent exposure to an allergen (e.g. peanuts), but more often it is either idiopathic (unknown) or only weakly correlated to allergen exposure.
In hereditary angioedema, often no direct cause is identifiable, although mild trauma, including dental work and other stimuli, can cause attacks.[4] There is usually no associated itch or urticaria, as it is not an allergic response. Patients with HAE can also have recurrent episodes (often called "attacks") of abdominal pain, usually accompanied by intense vomiting, weakness, and in some cases, watery diarrhea, and an unraised, nonitchy splotchy/swirly rash. These stomach attacks can last one to five days on average, and can require hospitalization for aggressive pain management and hydration. Abdominal attacks have also been known to cause a significant increase in the patient's white blood cell count, usually in the vicinity of 13,000 to 30,000. As the symptoms begin to diminish, the white count slowly begins to decrease, returning to normal when the attack subsides. As the symptoms and diagnostic tests are almost indistinguishable from an acute abdomen (e.g. perforated appendicitis) it is possible for undiagnosed HAE patients to undergo laparotomy (operations on the abdomen) or laparoscopy (keyhole surgery) that turns out to have been unnecessary.
HAE may also cause swelling in a variety of other locations, most commonly the limbs, genitals, neck, throat and face. The pain associated with these swellings varies from mildly uncomfortable to agonizing pain, depending on its location and severity. Predicting where and when the next episode of edema will occur is impossible. Most patients have an average of one episode per month, but there are also patients who have weekly episodes or only one or two episodes per year. The triggers can vary and include infections, minor injuries, mechanical irritation, operations or stress. In most cases, edema develops over a period of 12–36 hours and then subsides within 2–5 days.
诊断
The diagnosis is made on the clinical picture. Routine blood tests (complete blood count, electrolytes, renal function, liver enzymes) are typically performed. Mast cell tryptase levels may be elevated if the attack was due to an acute allergic (anaphylactic) reaction. When the patient has been stabilized, particular investigations may clarify the exact cause; complement levels, especially depletion of complement factors 2 and 4, may indicate deficiency of C1-inhibitor. HAE type III is a diagnosis of exclusion consisting of observed angioedema along with normal C1 levels and function.
The hereditary form (HAE) often goes undetected for a long time, as its symptoms resemble those of more common disorders, such as allergy or intestinal colic. An important clue is the failure of hereditary angioedema to respond to antihistamines or steroids, a characteristic that distinguishes it from allergic reactions. It is particularly difficult to diagnose HAE in patients whose episodes are confined to the gastrointestinal tract. Besides a family history of the disease, only a laboratory analysis can provide final confirmation. In this analysis, it is usually a reduced complement factor C4, rather than the C1-INH deficiency itself, that is detected. The former is used during the reaction cascade in the complement system of immune defense, which is permanently overactive due to the lack of regulation by C1-INH.
Angioedema is classified as either hereditary or acquired.
获得性血管性水肿
Acquired angioedema (AAE) can be immunologic, nonimmunologic, or idiopathic.[5] It is usually caused by allergy and occurs together with other allergic symptoms and urticaria. It can also occur as a side effect to certain medications, particularly ACE inhibitors. It is characterized by repetitive episodes of swelling, frequently of the face, lips, tongue, limbs, and genitals. Edema of the gastrointestinal mucosa typically leads to severe abdominal pain; in the upper respiratory tract, it can be life-threatening.[6]
遗传型血管性水肿
Hereditary angioedema (HAE) exists in three forms, all of which are caused by a genetic mutation inherited in an autosomal dominant form. They are distinguished by the underlying genetic abnormality. Types I and II are caused by mutations in the SERPING1 gene, which result in either diminished levels of the C1-inhibitor protein (type I HAE) or dysfunctional forms of the same protein (type II HAE). Type III HAE has been linked with mutations in the F12 gene, which encodes the coagulation protein factor XII. All forms of HAE lead to abnormal activation of the complement system, and all forms can cause swelling elsewhere in the body, such as the digestive tract. If HAE involves the larynx, it can cause life-threatening asphyxiation.[7] The pathogenesis of this disorder is suspected to be related to unopposed activation of the contact pathway by the initial generation of kallikrein and/or clotting factor XII by damaged endothelial cells. The end product of this cascade, bradykinin, is produced in large amounts and is believed to be the predominant mediator leading to increased vascular permeability and vasodilation that induces typical angioedema "attacks".[8]