title: Living related liver transplantation for polycystic liver disease.
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.
authors.affiliation: Present address: Department of Surgery, White 546, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114, USA. ichiro.koyama@tbrc.mgh.harvard.edu
authors.forename: Ichiro
authors.initials: I
authors.lastname: Koyama
authors.forename: Shohei
authors.initials: S
authors.lastname: Fuchinoue
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Urashima
authors.forename: Yojiro
authors.initials: Y
authors.lastname: Kato
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Tsuji
authors.forename: Tomonori
authors.initials: T
authors.lastname: Kawase
authors.forename: Toru
authors.initials: T
authors.lastname: Murakami
authors.forename: Tamotsu
authors.initials: T
authors.lastname: Tojimbara
authors.forename: Ichiro
authors.initials: I
authors.lastname: Nakajima
authors.forename: Satoshi
authors.initials: S
authors.lastname: Teraoka
mesh_terms: D000328:Adult; D003560:Cysts / Q000000981:diagnostic imaging / Q000601:surgery*; D005260:Female; D006801:Humans; D008107:Liver Diseases / Q000000981:diagnostic imaging / Q000601:surgery*; D016031:Liver Transplantation; D019520:Living Donors; D008297:Male; D008875:Middle Aged; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome