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

Research output: Contribution to journalArticle

4 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)1087-1091
Number of pages5
JournalSurgery Today
Volume35
Issue number12
DOIs
Publication statusPublished - 01-12-2005
Externally publishedYes

Fingerprint

Ornithine Carbamoyltransferase Deficiency Disease
Living Donors
Neuroimaging
Liver Transplantation
Quality of Life
Magnetic Resonance Imaging
Hyperammonemia
Hyperamylasemia
Lethargy
Brain Diseases
Frontal Lobe
Coma
Intelligence
Fathers
Renal Dialysis
Magnetic Resonance Spectroscopy
Tissue Donors

All Science Journal Classification (ASJC) codes

  • Surgery

Cite this

Kawagishi, Naoki ; Satoh, Kazushige ; Enomoto, Yoshitaka ; Akamatsu, Yorihiro ; Sekiguchi, Satoshi ; Satoh, Akira ; Fujimori, Keisei ; Takasago, Yuhei ; Ito, Tetsuya ; Ohura, Toshihiro ; Satomi, Susumu. / 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. In: Surgery Today. 2005 ; Vol. 35, No. 12. pp. 1087-1091.
@article{20c2355f3a63434383bfae961e0f2f19,
title = "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",
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.",
author = "Naoki Kawagishi and Kazushige Satoh and Yoshitaka Enomoto and Yorihiro Akamatsu and Satoshi Sekiguchi and Akira Satoh and Keisei Fujimori and Yuhei Takasago and Tetsuya Ito and Toshihiro Ohura and Susumu Satomi",
year = "2005",
month = "12",
day = "1",
doi = "10.1007/s00595-005-3071-y",
language = "English",
volume = "35",
pages = "1087--1091",
journal = "Surgery Today",
issn = "0941-1291",
publisher = "Springer Japan",
number = "12",

}

Kawagishi, N, Satoh, K, Enomoto, Y, Akamatsu, Y, Sekiguchi, S, Satoh, A, Fujimori, K, Takasago, Y, Ito, T, Ohura, T & Satomi, S 2005, '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', Surgery Today, vol. 35, no. 12, pp. 1087-1091. https://doi.org/10.1007/s00595-005-3071-y

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. / Kawagishi, Naoki; Satoh, Kazushige; Enomoto, Yoshitaka; Akamatsu, Yorihiro; Sekiguchi, Satoshi; Satoh, Akira; Fujimori, Keisei; Takasago, Yuhei; Ito, Tetsuya; Ohura, Toshihiro; Satomi, Susumu.

In: Surgery Today, Vol. 35, No. 12, 01.12.2005, p. 1087-1091.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Improved quality of life and unchanged magnetic resonance brain imaging after living donor liver transplantation for late-onset ornithine transcarbamylase deficiency

T2 - Report of a case

AU - Kawagishi, Naoki

AU - Satoh, Kazushige

AU - Enomoto, Yoshitaka

AU - Akamatsu, Yorihiro

AU - Sekiguchi, Satoshi

AU - Satoh, Akira

AU - Fujimori, Keisei

AU - Takasago, Yuhei

AU - Ito, Tetsuya

AU - Ohura, Toshihiro

AU - Satomi, Susumu

PY - 2005/12/1

Y1 - 2005/12/1

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=28844454272&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=28844454272&partnerID=8YFLogxK

U2 - 10.1007/s00595-005-3071-y

DO - 10.1007/s00595-005-3071-y

M3 - Article

C2 - 16341494

AN - SCOPUS:28844454272

VL - 35

SP - 1087

EP - 1091

JO - Surgery Today

JF - Surgery Today

SN - 0941-1291

IS - 12

ER -