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原发性胆汁性肝硬化肝移植术后新发自身免疫性肝炎。

De novo autoimmune hepatitis following liver transplantation for primary biliary cirrhosis.

作者信息

Rodríguez-Diaz Y, Reyes-Rodriguez R, Dorta-Francisco M C, Aguilera I, Perera-Molinero A, Moneva-Arce E, Aviles-Ruiz J F

机构信息

Servicios de Aparato Digestivo, Hospital Universitario N.S. de Candelaria, Islas Canarias, Sevilla, España.

出版信息

Transplant Proc. 2006 Jun;38(5):1467-70. doi: 10.1016/j.transproceed.2006.03.071.

DOI:10.1016/j.transproceed.2006.03.071
PMID:16797335
Abstract

De novo autoimmune hepatitis (AIH) is a rare disorder first described in 1998. It appears in patients with liver transplants for non-AIH etiology. The few cases in the literature make it a little known entity due to mechanisms that are unclear. We present the case of a woman with a liver transplant whose graft developed de novo AIH. Diagnosed with primary biliary cirrhosis, the patient underwent liver transplantation for refractory pruritus. Sixteen months after transplantation, we detected alterations in the hepatic profile with hypertransaminasemia and elevated alkaline phosphatase, together with elevated antinuclear antibodies. IgG levels were normal. Histological findings indicated AIH. The patient responded rapidly to treatment with prednisolone and azathioprine. De novo AIH in patients transplanted for PBC may cause graft dysfunction. This report also analyzes the case with respect to the other four reported cases, discussing etiologic hypotheses.

摘要

新发自身免疫性肝炎(AIH)是一种罕见疾病,于1998年首次被描述。它出现在因非AIH病因接受肝移植的患者中。由于发病机制尚不清楚,文献中报道的病例较少,这使得它成为一个鲜为人知的疾病实体。我们报告一例肝移植女性患者,其移植肝发生了新发AIH。该患者被诊断为原发性胆汁性肝硬化,因顽固性瘙痒接受了肝移植。移植后16个月,我们检测到肝功能指标异常,伴有转氨酶升高和碱性磷酸酶升高,同时抗核抗体升高。免疫球蛋白G水平正常。组织学检查结果提示为AIH。患者对泼尼松龙和硫唑嘌呤治疗反应迅速。因原发性胆汁性肝硬化接受移植的患者发生新发AIH可能导致移植肝功能障碍。本报告还结合其他4例报告病例对该病例进行了分析,并讨论了病因假说。

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World J Gastroenterol. 2018 Aug 7;24(29):3239-3249. doi: 10.3748/wjg.v24.i29.3239.
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De novo autoimmune hepatitis in liver transplant: State-of-the-art review.肝移植中的新发自身免疫性肝炎:最新综述
World J Gastroenterol. 2016 Mar 14;22(10):2906-14. doi: 10.3748/wjg.v22.i10.2906.
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Late liver function test abnormalities post-adult liver transplantation: a review of the etiology, investigation, and management.
成人肝移植术后晚期肝功能检查异常:病因、检查及管理综述
Hepatol Int. 2016 Jan;10(1):106-14. doi: 10.1007/s12072-015-9685-2. Epub 2015 Nov 24.
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Overlap syndromes: an emerging diagnostic and therapeutic challenge.重叠综合征:一项新出现的诊断和治疗挑战。
Saudi J Gastroenterol. 2014 Nov-Dec;20(6):342-9. doi: 10.4103/1319-3767.145316.
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Is there a role for cyclophilin inhibitors in the management of primary biliary cirrhosis?环孢素抑制剂在原发性胆汁性肝硬化治疗中的作用如何?
Viruses. 2013 Jan 24;5(2):423-38. doi: 10.3390/v5020423.
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