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A new technique for neonatal Jeune syndrome: External thoracic expansion

Jeune syndrome is a rare form of skeletal dysplasia characterized by a narrow, bell-shaped chest (thoracic cage), and typical phalangeal and pelvic bone deformities. Chest expansion is impaired by the short, horizontally positioned ribs, resulting in alveolar hypoventilation and eventually neonatal-...

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Detalles Bibliográficos
Autores principales: Temel, Uğur, Akgül, Aslı Gül
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Bayçınar Medical Publishing 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8167477/
https://www.ncbi.nlm.nih.gov/pubmed/34104526
http://dx.doi.org/10.5606/tgkdc.dergisi.2021.19731
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author Temel, Uğur
Akgül, Aslı Gül
author_facet Temel, Uğur
Akgül, Aslı Gül
author_sort Temel, Uğur
collection PubMed
description Jeune syndrome is a rare form of skeletal dysplasia characterized by a narrow, bell-shaped chest (thoracic cage), and typical phalangeal and pelvic bone deformities. Chest expansion is impaired by the short, horizontally positioned ribs, resulting in alveolar hypoventilation and eventually neonatal-infantile death in most cases. External distraction with sternoplasty is a new technique for the treatment of Jeune syndrome, which was firstly used by our team on a newborn by placing a sliding finger fixator which was designed for ulnar lengthening. We believe that this approach can be life-saving in neonates with improved and widespread usage.
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spelling pubmed-81674772021-06-07 A new technique for neonatal Jeune syndrome: External thoracic expansion Temel, Uğur Akgül, Aslı Gül Turk Gogus Kalp Damar Cerrahisi Derg Case Report Jeune syndrome is a rare form of skeletal dysplasia characterized by a narrow, bell-shaped chest (thoracic cage), and typical phalangeal and pelvic bone deformities. Chest expansion is impaired by the short, horizontally positioned ribs, resulting in alveolar hypoventilation and eventually neonatal-infantile death in most cases. External distraction with sternoplasty is a new technique for the treatment of Jeune syndrome, which was firstly used by our team on a newborn by placing a sliding finger fixator which was designed for ulnar lengthening. We believe that this approach can be life-saving in neonates with improved and widespread usage. Bayçınar Medical Publishing 2021-04-26 /pmc/articles/PMC8167477/ /pubmed/34104526 http://dx.doi.org/10.5606/tgkdc.dergisi.2021.19731 Text en Copyright © 2021, Turkish Society of Cardiovascular Surgery https://creativecommons.org/licenses/by-nc/4.0/This is an open access article under the terms of the Creative Commons Attribution-NonCommercial License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes.
spellingShingle Case Report
Temel, Uğur
Akgül, Aslı Gül
A new technique for neonatal Jeune syndrome: External thoracic expansion
title A new technique for neonatal Jeune syndrome: External thoracic expansion
title_full A new technique for neonatal Jeune syndrome: External thoracic expansion
title_fullStr A new technique for neonatal Jeune syndrome: External thoracic expansion
title_full_unstemmed A new technique for neonatal Jeune syndrome: External thoracic expansion
title_short A new technique for neonatal Jeune syndrome: External thoracic expansion
title_sort new technique for neonatal jeune syndrome: external thoracic expansion
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8167477/
https://www.ncbi.nlm.nih.gov/pubmed/34104526
http://dx.doi.org/10.5606/tgkdc.dergisi.2021.19731
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