Open AccessLiving related liver transplantation for polycystic liver disease
Author(s) -
Koyama Ichiro,
Fuchinoue Shohei,
Urashima Yoshinori,
Kato Yojiro,
Tsuji Kazuhiko,
Kawase Tomonori,
Murakami Toru,
Tojimbara Tamotsu,
Nakajima Ichiro,
Teraoka Satoshi
Publication year - 2002
Publication title -
transplant international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.998
H-Index - 82
eISSN - 1432-2277
pISSN - 0934-0874
DOI - 10.1111/j.1432-2277.2002.tb00111.x
Subject(s) - medicine , liver transplantation , polycystic liver disease , liver disease , polycystic disease , gastroenterology , polycystic kidney , transplantation , kidney disease , kidney
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 hepateciomy 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.