Talavera A, Larraona J L, Ramos J L, López T, Maraver A, Arias J, Barrios A
Department of Internal Medicine (Section of Gastroenterology and Hepatology), Hospital Infanta Elena, Huelva, Spain.
Am J Gastroenterol. 1995 Mar;90(3):471-4.
Patients with hereditary angioedema have episodes of local swelling, usually affecting the face, extremities, upper airway, and gastrointestinal tract. Only infrequently does it cause recurrent abdominal pain (with or without ascites); however, because it has potentially life-threatening complications, an early diagnosis is important. We describe a case of type I hereditary angioedema (a quantitative deficit of C1 inhibitor), the sole initial symptom of which was severe recurrent and self-limited abdominal pain, accompanied by ascites during these episodes. During a 4-yr period of treatment with danazol, the patient was virtually asymptomatic, despite levels of C4 and C1 inhibitor that remained below normal limits, and there have been no major side effects that could be attributed to treatment with androgens.
遗传性血管性水肿患者会出现局部肿胀发作，通常影响面部、四肢、上呼吸道和胃肠道。它很少引起反复腹痛（伴或不伴有腹水）；然而，由于其有潜在的危及生命的并发症，早期诊断很重要。我们描述一例I型遗传性血管性水肿（C1抑制剂定量缺乏）病例，其唯一的初始症状是严重的反复性自限性腹痛，发作期间伴有腹水。在使用达那唑治疗的4年期间，尽管C4和C1抑制剂水平仍低于正常范围，但患者几乎没有症状，且未出现可归因于雄激素治疗的重大副作用。
