Partial External Biliary Diversion Prevents Progression of Liver Fibrosis in Progressive Familial Intrahepatic Cholestasis

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  • Minagawa Nozomi
    First Department of Surgery, Hokkaido University Graduate School of Medicine
  • Sasaki Fumiaki
    Department of Pediatric Surgery, Hokkaido University Graduate School of Medicine
  • Okada Tadao
    Department of Pediatric Surgery, Hokkaido University Graduate School of Medicine
  • Kosugiyama Kiyotaka
    Department of Pediatrics, Hokkaido University Graduate School of Medicine
  • Kubota Mitsuru
    Department of Pediatrics, Hokkaido University Graduate School of Medicine
  • Ota Satoru
    Department of Pathology, Hokkaido University Graduate School of Medicine
  • Ito Tomoo
    Department of Pathology, Hokkaido University Graduate School of Medicine
  • Furukawa Hiroyuki
    Department of Transplantation, Hokkaido University Graduate School of Medicine
  • Noguchi Keita
    First Department of Surgery, Hokkaido University Graduate School of Medicine
  • Todo Satoru
    First Department of Surgery, Hokkaido University Graduate School of Medicine

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Other Title
  • 進行性家族性肝内胆汁うっ滞症に対し部分的胆汁外瘻術が肝線維化の進行阻止に有効であった1例
  • 症例報告 進行性家族性肝内胆汁うっ滞症に対し部分的胆汁外瘻術が肝線維化の進行阻止に有効であった1例
  • ショウレイ ホウコク シンコウセイ カゾクセイ カンナイ タンジュウウッタイショウ ニ タイシ ブブンテキ タンジュウ ガイロウジュツ ガ カン センイカ ノ シンコウ ソシ ニ ユウコウ デ アッタ 1レイ

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Progressive familial intrahepatic cholestasis (PFIC) is an inherited disorder of childhood in which cholestasis of hepatocellular origin often presents in the neonatal period. Our patient is a nine-year-old male child with PFIC who was admitted to our hospital to be evaluated for living-related liver transplantation. Our examination of the patient included a liver biopsy that revealed chronic cholestasis with a decreased γ-GTP level, confirming the diagnosis of PFIC (also known as Byler disease). Because we were unable to find a suitable donor for living-related liver transplantation, we opted for treatment with a high-dose choleretic agent, but liver fibrosis continued to worsen. In order to prevent liver cirrhosis, we performed a partial external biliary diversion (PEBD), which alleviated pruritus and improved liver function tests (eg, total bilirubin and total bile acid). One year after surgery, we performed an open liver biopsy that showed the fibrosis had not worsened. On the basis of our findings, we recommend that PEBD be performed in patients with PFIC refractory to medical management, before liver fibrosis progresses to an irreversible phase of cirrhosis.

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