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多囊性肝病的活体亲属肝移植

Living related liver transplantation for polycystic liver disease.

作者信息

Koyama Ichiro, Fuchinoue Shohei, Urashima Yoshinori, Kato Yojiro, Tsuji Kazuhiko, Kawase Tomonori, Murakami Toru, Tojimbara Tamotsu, Nakajima Ichiro, Teraoka Satoshi

机构信息

Present address: Department of Surgery, White 546, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114, USA.

出版信息

Transpl Int. 2002 Nov;15(11):578-80. doi: 10.1007/s00147-002-0459-0. Epub 2002 Sep 27.

Abstract

Polycystic liver disease (PCLD) is a rare inherited disorder, often associated with polycystic disease of the kidney. Although liver failure is unusual, some patients suffer from hepatic enlargement associated with severe complications such as abdominal distention, cachexia and dyspnea. Until recently, many surgical attempts had been made to reduce hepatic size, however, results have been unsatisfactory [3, 9, 10]. Today, liver transplantation is recommended as a therapeutic option, and excellent outcome has been demonstrated [1, 2, 4, 5, 6, 8, 11]. In this paper, we present the first case study of total hepatectomy and partial liver transplantation for PCLD, from a living, related donor. The patient is a 38-year-old man with PCLD who underwent living related liver transplantation (LRLT). He is alive and well 21 months after the operation, with complete resolution of the symptoms. He has returned to his previous job, with a marked improvement in his quality of life. Our experience demonstrates that LRLT can be an option for treatment of PCLD.

摘要

多囊肝病(PCLD)是一种罕见的遗传性疾病,常与多囊肾病相关。虽然肝衰竭并不常见,但一些患者会出现肝脏肿大,并伴有严重并发症,如腹胀、恶病质和呼吸困难。直到最近,人们进行了许多手术尝试来减小肝脏大小,然而,结果并不令人满意[3, 9, 10]。如今,肝移植被推荐为一种治疗选择,并且已证明有良好的效果[1, 2, 4, 5, 6, 8, 11]。在本文中,我们展示了首例来自活体亲属供体的全肝切除及部分肝移植治疗PCLD的病例研究。该患者是一名38岁的患有PCLD的男性,接受了活体亲属肝移植(LRLT)。术后21个月,他存活且状况良好,症状完全缓解。他已重返先前的工作岗位,生活质量有了显著改善。我们的经验表明,LRLT可以作为PCLD的一种治疗选择。

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