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原发性胆汁性肝硬化患者合并抗p97/VCP抗体且临床病程较轻的病例

[Case of primary biliary cirrhosis patient with anti-p97/VCP antibodies presenting a mild clinical course].

作者信息

Miyachi Kiyomitsu, Shibata Minoru, Hasegawa Chikako, Onozuka Yasushi, Fritzler Marvin J

机构信息

Keigu Clinic, Yokohama 230-0023.

出版信息

Rinsho Byori. 2005 Jan;53(1):19-23.

Abstract

In this study, we present a case of mild PBC that had anti-p97/VCP. A 53-year-old woman had been suspected of having chronic liver disease since 1983. In 1998, she visited the clinic, complaining of struma and pruritus. Laboratory findings on the first visit showed elevated levels of alkaline phosphatase (ALP) 454 IU/l(110-360), gammaGTP 250IU/l(-45) and IgM 671mg/dl(35-220). A screening of anti-mitochondrial antibody test was positive at a 1:80 dilution. A liver biopsy specimen revealed PBC at Scheuer stage 1. Following a treatment of ursodeoxycolic acid (UDCA) 300mg/day for 6 months, AMA and IgM were reduced to 1:20 and 220mg/dl, respectively. However, she was found to have low titer of anti-p97/VCP antibodies, determined by immunoprecipitation of radiolabeled recombinant protein produced by in vitro translation and transcription of the full length p97 cDNA. She has continued to be clinically stable following administration of UDCA 300mg. A PBC patient with anti-p97/VCP antibody showed a milder clinical course, suggesting some beneficial role of this antibody.

摘要

在本研究中,我们报告了一例患有抗p97/VCP的轻度原发性胆汁性胆管炎(PBC)病例。一名53岁女性自1983年起就被怀疑患有慢性肝病。1998年,她前往诊所就诊,主诉甲状腺肿和瘙痒。首次就诊时的实验室检查结果显示碱性磷酸酶(ALP)水平升高至454 IU/l(110 - 360),γ-谷氨酰转肽酶(gammaGTP)为250 IU/l(-45),免疫球蛋白M(IgM)为671 mg/dl(35 - 220)。抗线粒体抗体检测筛查在1:80稀释度时呈阳性。肝活检标本显示为Scheuer 1期的原发性胆汁性胆管炎。在接受熊去氧胆酸(UDCA)300mg/天治疗6个月后,抗线粒体抗体(AMA)和IgM分别降至1:20和220mg/dl。然而,通过对全长p97 cDNA进行体外翻译和转录产生的放射性标记重组蛋白进行免疫沉淀测定,发现她具有低滴度的抗p97/VCP抗体。在给予300mg UDCA后,她的病情持续保持临床稳定。一名患有抗p97/VCP抗体的原发性胆汁性胆管炎患者临床病程较轻,提示该抗体具有一定的有益作用。

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