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Congential scoliosis in Wilson’s disease: case report and review of the literature

BACKGROUND: Wilson’s disease (WD) is an autosomal recessive disorder of copper metabolism, which leads to the accumulation of this metal in liver, brain, cornea and kidney. Little is reported about spinal deformity associated with this syndrome. This study is to present a case of thoracic kyphosis o...

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Autores principales: Li, Zheng, Yu, Xin, Shen, Jianxiong, Liang, Jinqian
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4177382/
https://www.ncbi.nlm.nih.gov/pubmed/25252715
http://dx.doi.org/10.1186/1471-2482-14-71
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author Li, Zheng
Yu, Xin
Shen, Jianxiong
Liang, Jinqian
author_facet Li, Zheng
Yu, Xin
Shen, Jianxiong
Liang, Jinqian
author_sort Li, Zheng
collection PubMed
description BACKGROUND: Wilson’s disease (WD) is an autosomal recessive disorder of copper metabolism, which leads to the accumulation of this metal in liver, brain, cornea and kidney. Little is reported about spinal deformity associated with this syndrome. This study is to present a case of thoracic kyphosis occurring in the setting of Wilson’disease and explore the possible association between the two diseases. CASE PRESENTATION: Case report and literature review. A previously unreported thoracic kyphosis in Wislon’s disease is decribed. The patient was a 7-year-old Chinese female that underwent a posterior correction, using the Moss-SI spinal system performed at Thoracic 9-Lumbar 1 (T9-L1) levels. At 16-month follow-up, the patient was clinically pain free and well balanced. Plain radiographs showed solid spine fusion with no loss of deformity correction. After evaluating 211 WD patients referred to Peking Union Medical College Hospital from February 1991 to February 2012, the prevalence of congential scoliosis among patients with WD was 5.21% (11/211), much higher than that among general population (1/1000). CONCLUSIONS: To the best of our knowledge, this is the first report of WD with thoracic kyphosis. During sugery, surgeons and anesthesiologists must pay particular attention to the abnormal liver and brain function associated with WD. The prevalence of scoliosis is much higher among patients with WD, indicating a potential association between congential scoliosis and WD. However, the exact mechanism how copper-chelating agents induce scoliosis is unclear.
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spelling pubmed-41773822014-09-29 Congential scoliosis in Wilson’s disease: case report and review of the literature Li, Zheng Yu, Xin Shen, Jianxiong Liang, Jinqian BMC Surg Case Report BACKGROUND: Wilson’s disease (WD) is an autosomal recessive disorder of copper metabolism, which leads to the accumulation of this metal in liver, brain, cornea and kidney. Little is reported about spinal deformity associated with this syndrome. This study is to present a case of thoracic kyphosis occurring in the setting of Wilson’disease and explore the possible association between the two diseases. CASE PRESENTATION: Case report and literature review. A previously unreported thoracic kyphosis in Wislon’s disease is decribed. The patient was a 7-year-old Chinese female that underwent a posterior correction, using the Moss-SI spinal system performed at Thoracic 9-Lumbar 1 (T9-L1) levels. At 16-month follow-up, the patient was clinically pain free and well balanced. Plain radiographs showed solid spine fusion with no loss of deformity correction. After evaluating 211 WD patients referred to Peking Union Medical College Hospital from February 1991 to February 2012, the prevalence of congential scoliosis among patients with WD was 5.21% (11/211), much higher than that among general population (1/1000). CONCLUSIONS: To the best of our knowledge, this is the first report of WD with thoracic kyphosis. During sugery, surgeons and anesthesiologists must pay particular attention to the abnormal liver and brain function associated with WD. The prevalence of scoliosis is much higher among patients with WD, indicating a potential association between congential scoliosis and WD. However, the exact mechanism how copper-chelating agents induce scoliosis is unclear. BioMed Central 2014-09-24 /pmc/articles/PMC4177382/ /pubmed/25252715 http://dx.doi.org/10.1186/1471-2482-14-71 Text en Copyright © 2014 Li et al.; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/4.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Li, Zheng
Yu, Xin
Shen, Jianxiong
Liang, Jinqian
Congential scoliosis in Wilson’s disease: case report and review of the literature
title Congential scoliosis in Wilson’s disease: case report and review of the literature
title_full Congential scoliosis in Wilson’s disease: case report and review of the literature
title_fullStr Congential scoliosis in Wilson’s disease: case report and review of the literature
title_full_unstemmed Congential scoliosis in Wilson’s disease: case report and review of the literature
title_short Congential scoliosis in Wilson’s disease: case report and review of the literature
title_sort congential scoliosis in wilson’s disease: case report and review of the literature
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4177382/
https://www.ncbi.nlm.nih.gov/pubmed/25252715
http://dx.doi.org/10.1186/1471-2482-14-71
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