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[C1抑制剂缺乏所致先天性血管性水肿患者的复发性腹痛和腹水。回顾性分析]

[Recurrent abdominal pain and ascites in patients suffering from congenital angioedema due to C1 inhibitor deficiency. Retrospective analysis].

作者信息

Obtułowicz Piotr, Urbanik Andrzej, Obtutłowicz Krystyna

机构信息

Katedra Radiologii, Uniwersytet Jagielloński, Collegium Medicum.

出版信息

Przegl Lek. 2013;70(5):299-302.

Abstract

The aim of the study was the estimation of diagnostic value abdominal ultrasonography in recurrent attacks the patients suffering from hereditary angioedema. The retrospective analysis was done in 150 patients with C1 inhibitor deficiency and abdomen attack of angioedema connected with this. In 55 patients severe 2-3 days longlasting abdomen attacks with nausea, diarrhoea and weakening were the first symptoms of the illness and causing particular diagnostic problem. In 95 remaining patients abdomen attack appeared in the course of illness manifesting external angioedema of hands, feets, face, genitalia or larynx. The frequency of abdominal attacks was different from 1-15/year. Diagnostic problem was the reason of laparotomy in 19.3% of patients which did not explain the reason of symptoms. Only in 23 % of patients US of abdomen was done, which revealed the presence of ascites, which disappeared together with abdomen symptoms. US often allowed to show the place of regional angioedema usually in the most cases in intestine wall.

摘要

本研究的目的是评估腹部超声检查对遗传性血管性水肿患者反复发作的诊断价值。对150例C1抑制剂缺乏且伴有血管性水肿腹部发作的患者进行了回顾性分析。55例患者中,严重的、持续2 - 3天的腹部发作伴有恶心、腹泻和虚弱是疾病的首发症状,造成了特殊的诊断难题。其余95例患者在病程中出现腹部发作,表现为手部、足部、面部、生殖器或喉部的外部血管性水肿。腹部发作的频率为每年1 - 15次不等。19.3%的患者因诊断问题接受了剖腹手术,但未查明症状原因。仅23%的患者进行了腹部超声检查,结果显示存在腹水,腹水随着腹部症状的消失而消失。超声检查通常能显示局部血管性水肿的部位,大多数情况下位于肠壁。

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