反复腹痛发作;遗传性血管性水肿

E. Emre, A. Kan
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摘要

遗传性血管性水肿(遗传性血管性水肿,遗传性血管性水肿)是一种重要的疾病,其特征是反复发作的血管性水肿而无荨麻疹,最常影响上呼吸道、泌尿生殖系统和胃肠道的皮肤或粘膜组织。血管性水肿大多是自限性的,无需治疗即可在几天内消退,但在某些情况下,如上呼吸道受累,可能导致致命的窒息。大多数HAE患者都经历过胃肠道发作,并且可能是主要表现。遗传性血管性水肿的诊断在许多病例中可能会延迟。许多未确诊的HAE患者可能会进行不适当的腹部手术。在这项研究中,我们报告了一名14岁的患者和他的母亲,他们被诊断为1型HAE,主要是由于肠血管性水肿引起的腹痛发作。患者因不明原因腹痛,先后行阑尾切除术和两次诊断性剖腹手术。通过这一病例报告,我们希望提高医生对HAE的认识。
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Recurrent Abdominal Pain Attacks; Hereditary Angioedema
ABS TRACT Hereditary angioedema (HAE) is an important disease characterized by recurrent episodes of angioedema without urticaria, which most often affects the skin or mucosal tissues of the upper respiratory, genitourinary and gastrointestinal tracts. The angioedema is mostly self-limited and resolves in a few days without treatment, however in some conditions such as upper respiratory involvement, may cause fatal asphyxiation. Gastrointestinal attacks are experienced by a majority of patients with HAE and can be the principal presentation. The diagnosis of hereditary angioedema may be delayed in many cases. Many patients with undiagnosed HAE may undergo inappropriate abdominal surgery. In this study, we are presenting a 14 years-old patient and his mother who are diagnosed with type 1 HAE, predominantly suffering from abdominal pain attacks due to intestinal angioedema. The patient underwent appendectomy and two times diagnostic laparotomy at different times due to unexplained abdominal pain. With this case report we wanted to raise awareness of physicians about HAE.
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