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

研究成果: Article査読

4 被引用数 (Scopus)

抄録

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.

本文言語English
ページ(範囲)1087-1091
ページ数5
ジャーナルSurgery Today
35
12
DOI
出版ステータスPublished - 12-2005
外部発表はい

All Science Journal Classification (ASJC) codes

  • 外科

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