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Anesthetic Management of a Pediatric Patient With Wilsons Disease
Wilsons disease, characterized by cirrhosis, extrapyramidal symptoms and Kayser-Fleischer corneal rings, is a rare hereditary disease of human copper metabolism. Clinical findings in Wilsons disease are complex and neurological symptoms such as tremor, dysarthria, rigid dystonia, seizures, psychiatr...
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elmer Press
2010
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3140888/ https://www.ncbi.nlm.nih.gov/pubmed/21811529 http://dx.doi.org/10.4021/jocmr2010.04.292w |
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author | Baykal, Mehmet Karapolat, Sami |
author_facet | Baykal, Mehmet Karapolat, Sami |
author_sort | Baykal, Mehmet |
collection | PubMed |
description | Wilsons disease, characterized by cirrhosis, extrapyramidal symptoms and Kayser-Fleischer corneal rings, is a rare hereditary disease of human copper metabolism. Clinical findings in Wilsons disease are complex and neurological symptoms such as tremor, dysarthria, rigid dystonia, seizures, psychiatric disorders, acute liver failure, chronic hepatitis or cirrhosis may develop. A 4-year-old male patient was operated for traumatic depressed skull fracture and intracerebral hematoma. He was diagnosed with Wilsons disease at the age of 2.5 years and treated with zinc sulphate and D-penicillamine. General anesthesia was induced with propofol, fentanyl, atracurium, and maintained with isoflurane, and oxygen. No complications were encountered during the operation or in the postoperative period. We concluded that general anesthesia can successfully be given to Wilsons disease patients using an anesthetic agent, the metabolism of which is least affected by the liver disease, one that induces least hepatic toxicity. By close follow-up of patients clinically and biochemically, it is possible to reduce the complication rates to a minimum. KEYWORDS: Wilson's Disease; Craniocerebral trauma; Thoracic injuries; General anesthesia; Surgery |
format | Online Article Text |
id | pubmed-3140888 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2010 |
publisher | Elmer Press |
record_format | MEDLINE/PubMed |
spelling | pubmed-31408882011-08-02 Anesthetic Management of a Pediatric Patient With Wilsons Disease Baykal, Mehmet Karapolat, Sami J Clin Med Res Case Report Wilsons disease, characterized by cirrhosis, extrapyramidal symptoms and Kayser-Fleischer corneal rings, is a rare hereditary disease of human copper metabolism. Clinical findings in Wilsons disease are complex and neurological symptoms such as tremor, dysarthria, rigid dystonia, seizures, psychiatric disorders, acute liver failure, chronic hepatitis or cirrhosis may develop. A 4-year-old male patient was operated for traumatic depressed skull fracture and intracerebral hematoma. He was diagnosed with Wilsons disease at the age of 2.5 years and treated with zinc sulphate and D-penicillamine. General anesthesia was induced with propofol, fentanyl, atracurium, and maintained with isoflurane, and oxygen. No complications were encountered during the operation or in the postoperative period. We concluded that general anesthesia can successfully be given to Wilsons disease patients using an anesthetic agent, the metabolism of which is least affected by the liver disease, one that induces least hepatic toxicity. By close follow-up of patients clinically and biochemically, it is possible to reduce the complication rates to a minimum. KEYWORDS: Wilson's Disease; Craniocerebral trauma; Thoracic injuries; General anesthesia; Surgery Elmer Press 2010-04 2010-04-15 /pmc/articles/PMC3140888/ /pubmed/21811529 http://dx.doi.org/10.4021/jocmr2010.04.292w Text en Copyright © 2010, Baykal et al. http://creativecommons.org/licenses/by/2.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Baykal, Mehmet Karapolat, Sami Anesthetic Management of a Pediatric Patient With Wilsons Disease |
title | Anesthetic Management of a Pediatric Patient With Wilsons Disease |
title_full | Anesthetic Management of a Pediatric Patient With Wilsons Disease |
title_fullStr | Anesthetic Management of a Pediatric Patient With Wilsons Disease |
title_full_unstemmed | Anesthetic Management of a Pediatric Patient With Wilsons Disease |
title_short | Anesthetic Management of a Pediatric Patient With Wilsons Disease |
title_sort | anesthetic management of a pediatric patient with wilsons disease |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3140888/ https://www.ncbi.nlm.nih.gov/pubmed/21811529 http://dx.doi.org/10.4021/jocmr2010.04.292w |
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