Surgery Today

, Volume 35, Issue 12, pp 1087–1091

Improved Quality of Life and Unchanged Magnetic Resonance Brain Imaging After Living Donor Liver Transplantation for Late-Onset Ornithine Transcarbamylase Deficiency: Report of a Case

  • Naoki Kawagishi
  • Kazushige Satoh
  • Yoshitaka Enomoto
  • Yorihiro Akamatsu
  • Satoshi Sekiguchi
  • Akira Satoh
  • Keisei Fujimori
  • Yuhei Takasago
  • Tetsuya Ito
  • Toshihiro Ohura
  • Susumu Satomi
Article

DOI: 10.1007/s00595-005-3071-y

Cite this article as:
Kawagishi, N., Satoh, K., Enomoto, Y. et al. Surg Today (2005) 35: 1087. doi:10.1007/s00595-005-3071-y

Abstract

We report the case of a 7-year-old girl with ornithine transcarbamylase deficiency whose quality of life (QOL) improved greatly after a living donor liver transplantation (LDLT). Ornithine transcarbamylase deficiency had been diagnosed when she was 2 years old and she finally underwent LDLT, with her father as the donor, when she was 7 years old. The patient had suffered episodes of hyperammonemic encephalopathy ranging from lethargy to coma, treated by hemodialysis twice before LDLT, and her intelligence quotient was borderline for her age. Preoperative magnetic resonance imaging (MRI) showed an atrophic area in the subcortical white matter of the frontal lobe. After LDLT, the patient suffered acute rejection with hyperamylasemia, but not hyperammonemia. Postoperative MRI and quantitative MR spectroscopy showed no changes in the subcortical lesion. She has been followed up carefully for 16 months and has had no further complications or any sign of hyperammonemia.

Key words

Living donor liver transplantation Ornithine transcarbamylase deficiency Magnetic resonance imaging Brain atrophy Quality of life 

Copyright information

© Springer-Verlag Tokyo 2005

Authors and Affiliations

  • Naoki Kawagishi
    • 1
  • Kazushige Satoh
    • 1
  • Yoshitaka Enomoto
    • 1
  • Yorihiro Akamatsu
    • 1
  • Satoshi Sekiguchi
    • 1
  • Akira Satoh
    • 1
  • Keisei Fujimori
    • 1
  • Yuhei Takasago
    • 2
  • Tetsuya Ito
    • 3
  • Toshihiro Ohura
    • 4
  • Susumu Satomi
    • 1
  1. 1.Division of Advanced Surgical Science and TechnologyTohoku UniversitySendaiJapan
  2. 2.Pediatric Department, Morioka Children's HospitalMoriokaJapan
  3. 3.Department of PediatricsNagoya City University Medical SchoolNagoyaJapan
  4. 4.Department of Pediatrics, Graduate School of MedicineTohoku UniversitySendaiJapan

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