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Anesthetic Approach for a Patient with Jeune Syndrome

Jeune syndrome (JS) is an autosomal recessive disease also known as asphyxiating thoracic dystrophy. A narrow bell-shaped thoracic wall and short extremities are the most typical features of the syndrome. Prognosis in JS depends on the severity of the pulmonary hypoplasia caused by the chest wall de...

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Detalles Bibliográficos
Autores principales: Buget, Mehmet I., Ozkan, Emine, Edipoglu, Ipek S., Kucukay, Suleyman
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4561095/
https://www.ncbi.nlm.nih.gov/pubmed/26366306
http://dx.doi.org/10.1155/2015/509196
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author Buget, Mehmet I.
Ozkan, Emine
Edipoglu, Ipek S.
Kucukay, Suleyman
author_facet Buget, Mehmet I.
Ozkan, Emine
Edipoglu, Ipek S.
Kucukay, Suleyman
author_sort Buget, Mehmet I.
collection PubMed
description Jeune syndrome (JS) is an autosomal recessive disease also known as asphyxiating thoracic dystrophy. A narrow bell-shaped thoracic wall and short extremities are the most typical features of the syndrome. Prognosis in JS depends on the severity of the pulmonary hypoplasia caused by the chest wall deformity. Most patient deaths are due to respiratory problems at early ages. Herein, we report a case of JS patient, who was scheduled for femoral extension under general anesthesia. The severity of respiratory problems in JS patients is thought to diminish with age. Our case supported this theory, and we managed the anesthetic process uneventfully.
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spelling pubmed-45610952015-09-13 Anesthetic Approach for a Patient with Jeune Syndrome Buget, Mehmet I. Ozkan, Emine Edipoglu, Ipek S. Kucukay, Suleyman Case Rep Anesthesiol Case Report Jeune syndrome (JS) is an autosomal recessive disease also known as asphyxiating thoracic dystrophy. A narrow bell-shaped thoracic wall and short extremities are the most typical features of the syndrome. Prognosis in JS depends on the severity of the pulmonary hypoplasia caused by the chest wall deformity. Most patient deaths are due to respiratory problems at early ages. Herein, we report a case of JS patient, who was scheduled for femoral extension under general anesthesia. The severity of respiratory problems in JS patients is thought to diminish with age. Our case supported this theory, and we managed the anesthetic process uneventfully. Hindawi Publishing Corporation 2015 2015-08-23 /pmc/articles/PMC4561095/ /pubmed/26366306 http://dx.doi.org/10.1155/2015/509196 Text en Copyright © 2015 Mehmet I. Buget et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under 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
Buget, Mehmet I.
Ozkan, Emine
Edipoglu, Ipek S.
Kucukay, Suleyman
Anesthetic Approach for a Patient with Jeune Syndrome
title Anesthetic Approach for a Patient with Jeune Syndrome
title_full Anesthetic Approach for a Patient with Jeune Syndrome
title_fullStr Anesthetic Approach for a Patient with Jeune Syndrome
title_full_unstemmed Anesthetic Approach for a Patient with Jeune Syndrome
title_short Anesthetic Approach for a Patient with Jeune Syndrome
title_sort anesthetic approach for a patient with jeune syndrome
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4561095/
https://www.ncbi.nlm.nih.gov/pubmed/26366306
http://dx.doi.org/10.1155/2015/509196
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