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Hereditary angioedema: A rare cause of recurrent abdominal pain

机译:遗传性血管性水肿:罕见的复发性腹痛原因

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摘要

Hereditary angioedema is a rare autosomal dominant inherited disease which is characterized by an episodic, self-limiting increase in vascular permeability. Symptoms commonly involve in nonpitting, nonpruritic skin swellings. We present a case of hereditary angioedema. The patinets complained of a recurrent abdominal pain without accompanying skin swelling whose diagnosis was delayed nearly 20 years and accepted an unnecessary surgery. According to the decreased serum C1-inhibitor and C4 concentration, the patient was finally diagnosed with hereditary angioedema type I. After treatment with danazole, the patient reported a significant decrease in the frequency of attacks and the severity of pain. HAE is a rare cause of abdominal pain, however it needs to be taken as one of the differential diagnosis of various acute abdomens in order to avoid unnecessary surgeries.
机译:遗传性血管性水肿是一种罕见的常染色体显性遗传疾病,其特征是血管通透性的自限性增加。症状通常涉及无斑点,无瘙痒的皮肤肿胀。我们提出一例遗传性血管性水肿。该小片抱怨腹部疼痛反复发作而没有皮肤肿胀,其诊断被推迟了近20年,并接受了不必要的手术。根据降低的血清C1抑制剂和C4浓度,患者最终被确诊为I型遗传性血管性水肿。用达那唑治疗后,患者报告发作频率和疼痛严重程度明显降低。 HAE是一种罕见的腹痛原因,但是为了避免不必要的手术,它必须作为各种急性腹部的鉴别诊断之一。

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