A Case of Mitochondrial DNA Depletion Syndrome With Hepatocellular Carcinoma in the Explanted Native Liver During Living-Donor Liver Transplantation

DOI
  • Uchida Yasuyuki
    Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University
  • Matsuura Toshiharu
    Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University
  • Kajihara Keisuke
    Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University
  • Maeda Shohei
    Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University
  • Toriigahara Yukihiro
    Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University
  • Tamaki Akihiko
    Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University
  • Takahashi Yoshiaki
    Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University
  • Tajiri Tatsuro
    Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University

Bibliographic Information

Other Title
  • 生体肝移植時の摘出肝に肝細胞癌を認めたミトコンドリアDNA枯渇症候群の1例

Description

<p>MPV17-related mitochondrial DNA depletion syndrome has a poor prognosis; nonetheless, depending on the mutated gene, there have been some cases of survival after liver transplantation with few neurological complications. However, there have been reports of a poor prognosis after liver transplantation in patients with neurological disorders prior to the procedure. Therefore, the indications for liver transplantation remain controversial. The patient was a 14-year-old girl. She was diagnosed as having MPV17-related mitochondrial DNA depletion syndrome at the age of 3 years on the basis of the liver biopsy finding of decreased mitochondrial DNA levels and the skin biopsy findings of c.293C>T and c376-1G>A gene mutations. Living donor liver transplantation from her father was performed for decompensated cirrhosis at 14 years of age. Hepatocellular carcinoma was incidentally detected in the explanted liver. Two years after liver transplantation, there was no evidence of rejection, liver dysfunction, or recurrence of hepatocellular carcinoma. However, her neurological symptoms gradually worsened. Careful consideration of the indications for liver transplantation in each case and further investigation of life and neurological prognoses after liver transplantation are needed.</p>

Journal

Keywords

Details 詳細情報について

  • CRID
    1390301919372741376
  • DOI
    10.11164/jjsps.60.6_949
  • ISSN
    21874247
    0288609X
  • Text Lang
    ja
  • Data Source
    • JaLC
  • Abstract License Flag
    Disallowed

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