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两例伴有阵发性腹痛的C1酯酶抑制剂缺乏症

[Two cases of C1INH deficiency with paroxysmal abdominal pain].

作者信息

Takamura Kazuto, Arai Keiko, Otsu Naoya

机构信息

Department of Internal Medicine, Fukushima Co-op Hospital.

出版信息

Nihon Shokakibyo Gakkai Zasshi. 2007 Jun;104(6):799-803.

PMID:17548946
Abstract

We encountered two cases of C1 inhibitor deficiency (26-year-old man and 29-year-old woman). They had been suffering from paroxysmal abdominal pain for many years. Imaging studies showed wall thickening of the intestine and ascites and diagnosis was difficult. Decreased serum levels of C4 and C1INH activity indicated a diagnosis of C1INH deficiency. Although C1INH deficiency is rare, it should be considered as a differential diagnosis in young patients with paroxysmal abdominal pain.

摘要

我们遇到了两例C1抑制剂缺乏症患者(一名26岁男性和一名29岁女性)。他们多年来一直遭受阵发性腹痛。影像学检查显示肠道壁增厚和腹水,诊断困难。血清C4水平降低和C1INH活性降低提示C1INH缺乏症的诊断。虽然C1INH缺乏症罕见,但在患有阵发性腹痛的年轻患者中应考虑将其作为鉴别诊断。

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