Fuse Takashi, Nakada Taka-aki, Taniguchi Masashi, Mizushima Yasuaki, Matsuoka Tetsuya
Senshu Trauma and Critical Care Center, 2-23 Rinku Orai Kita, Osaka 598-8577, Japan.
Senshu Trauma and Critical Care Center, 2-23 Rinku Orai Kita, Osaka 598-8577, Japan; Chiba University Graduate School of Medicine, 1-8-1 Inohana, Chuo, Chiba 260-8677, Japan.
Am J Emerg Med. 2015 Dec;33(12):1840.e1-2. doi: 10.1016/j.ajem.2015.04.003. Epub 2015 Apr 7.
Hereditary angioedema (HAE) is a rare genetic disease caused by a deficiency of functional C1 esterase inhibitor that causes swelling attacks in various body tissues. We hereby report a case of out-of-hospital cardiac arrest due to airway obstruction in HAE. Cutaneous swelling and abdominal pain attacks caused by gastrointestinal wall swelling are common symptoms in HAE, whereas laryngeal swelling is rare. Emergency physicians may have few chances to experience cases of life-threatening laryngeal edema resulting in a delay from symptom onset to the diagnosis of HAE. Hereditary angioedema is diagnosed by performing complement blood tests. Because safe and effective treatment options are available for the life-threatening swellings in HAE, the diagnosis potentially reduces the risk of asphyxiation in patients and their blood relatives.
遗传性血管性水肿(HAE)是一种罕见的遗传性疾病,由功能性C1酯酶抑制剂缺乏引起,可导致身体各组织出现肿胀发作。我们在此报告一例HAE患者因气道阻塞导致院外心脏骤停的病例。胃肠道壁肿胀引起的皮肤肿胀和腹痛发作是HAE的常见症状,而喉部肿胀则较为罕见。急诊医生很少有机会遇到危及生命的喉水肿病例,从而导致从症状出现到HAE诊断的延迟。遗传性血管性水肿通过进行补体血液检查来诊断。由于有安全有效的治疗方案可用于治疗HAE中危及生命的肿胀,该诊断有可能降低患者及其血亲窒息的风险。