The status of polycystic liver disease in Japan: a questionnaire survey of patients

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  • Ogawa Koichi
    Department of Surgery, Doctoral Program in Clinical Science, Graduate School of Comprehensive Human Sciences, University of Tsukuba
  • Fukunaga Kiyoshi
    Department of Surgery, Doctoral Program in Clinical Science, Graduate School of Comprehensive Human Sciences, University of Tsukuba
  • Takeuchi Tomoyo
    Institute of Clinical Medicine, Graduate School of Comprehensive Human Sciences, University of Tsukuba
  • Kawagishi Naoki
    Division of Organ Transplantation, Tohoku University Hospital
  • Kudo Masatoshi
    Department of Gastroenterology and Hepatology, Kinki University School of Medicine
  • Ohkouchi Nobuhiro
    Department of Surgery, Doctoral Program in Clinical Science, Graduate School of Comprehensive Human Sciences, University of Tsukuba

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Other Title
  • 本邦における多発肝嚢胞症のアンケート調査
  • ホンポウ ニ オケル タハツ ギモノウホウショウ ノ アンケート チョウサ

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Description

Polycystic liver disease (PLD) is genetic disorder characterized by the progressive development of multiple liver cysts. Because of rarity of PLD, therapeutic consensus is controversial. The aim of this study was to investigate a situation of PLD treatment by a questionnaire survey. In this study, 422 patients with PLD participated, and 223 patients underwent treatment. The most common symptom was abdominal distension (73%). The treatments were performed as follows; percutaneous aspiration in 27% of patients, liver resection in 12%, cyst fenestration in 9%, and liver transplantation in 3%. The efficacies of these treatments were recognized in 77%, 96%, 92%, and 100% of each patient, respectively. Because of no effect of primary therapy, additional treatment was performed in only 4.3% of patients. In general, the choice of primary treatment for PLD was considered reasonable and proper, in Japan.<br>

Journal

  • Kanzo

    Kanzo 52 (11), 709-715, 2011

    The Japan Society of Hepatology

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