Document Detail


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.
MedLine Citation:
PMID:  16341494     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Naoki Kawagishi; Kazushige Satoh; Yoshitaka Enomoto; Yorihiro Akamatsu; Satoshi Sekiguchi; Akira Satoh; Keisei Fujimori; Yuhei Takasago; Tetsuya Ito; Toshihiro Ohura; Susumu Satomi
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Surgery today     Volume:  35     ISSN:  0941-1291     ISO Abbreviation:  Surg. Today     Publication Date:  2005  
Date Detail:
Created Date:  2005-12-12     Completed Date:  2006-03-23     Revised Date:  2006-08-03    
Medline Journal Info:
Nlm Unique ID:  9204360     Medline TA:  Surg Today     Country:  Japan    
Other Details:
Languages:  eng     Pagination:  1087-91     Citation Subset:  IM    
Affiliation:
Division of Advanced Surgical Science and Technology, Tohoku University, 1-1 Seiryo-machi, Sendai, 980-8574, Japan.
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MeSH Terms
Descriptor/Qualifier:
Atrophy
Brain / pathology*
Child
Female
Humans
Liver Transplantation*
Living Donors
Magnetic Resonance Imaging
Ornithine Carbamoyltransferase Deficiency Disease / surgery*
Quality of Life*

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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