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一名患有常染色体显性多囊肾病的维持性血液透析患者,因难治性肝囊肿感染而单独接受了活体供肝移植。

A case of a maintenance hemodialysis patient with autosomal dominant polycystic kidney disease who underwent living donor liver transplantation alone due to refractory liver cyst infection.

作者信息

Akihisa Taro, Ino Ayami, Egawa Hiroto, Kotera Yoshihito, Ariizumi Shunichi, Oomori Akiko, Yamashita Shingo, Yamamoto Yusuke, Tsuchiya Ken, Yamamoto Masakazu, Nitta Kosaku, Mochizuki Toshio

机构信息

Department of Medicine, Kidney Center, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo, 162-8666, Japan.

Department of Surgery, Institute of Gastroenterology, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo, 162-8666, Japan.

出版信息

CEN Case Rep. 2018 Nov;7(2):307-312. doi: 10.1007/s13730-018-0348-8. Epub 2018 Jun 28.

Abstract

Liver cysts are observed in 83% of cases of autosomal dominant polycystic kidney disease (ADPKD). Although not as prevalent as renal cyst infection, liver cyst infection is a serious complication that is sometimes difficult to treat. We report the case of a maintenance hemodialysis patient with ADPKD who received a living donor liver transplantation alone (LDLTA) due to refractory liver cyst infection. The patient was a 67-year-old Japanese man who developed fever and right-side abdominal pain, and liver cyst infection was suspected. Treatment with multiple antibiotics was ineffective. Many liver cysts were observed on magnetic resonance imaging scans and a cyst in liver segment S6, which produced the strongest signal variation, was drained. The fever subsided temporarily, but multiple infected liver cysts were observed on follow-up imaging examination; 4 months later, hepatectomy and LDLTA were performed. Although LDLTA due to refractory liver cyst infection in maintenance hemodialysis patients with ADPKD is risky and should be carefully considered, it may be the only effective treatment.

摘要

在常染色体显性遗传性多囊肾病(ADPKD)病例中,83%会出现肝囊肿。尽管肝囊肿感染不如肾囊肿感染常见,但它是一种严重的并发症,有时难以治疗。我们报告了一例维持性血液透析的ADPKD患者,该患者因难治性肝囊肿感染接受了单纯活体供肝移植(LDLTA)。患者为一名67岁的日本男性,出现发热和右侧腹痛,怀疑有肝囊肿感染。使用多种抗生素治疗无效。磁共振成像扫描发现许多肝囊肿,对肝S6段产生最强信号变化的一个囊肿进行了引流。发热暂时消退,但在后续影像学检查中发现多个感染性肝囊肿;4个月后,进行了肝切除术和LDLTA。尽管对于维持性血液透析的ADPKD患者,因难治性肝囊肿感染进行LDLTA风险较大,应谨慎考虑,但这可能是唯一有效的治疗方法。

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