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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-...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Bayçınar Medical Publishing
2021
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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. |
format | Online Article Text |
id | pubmed-8167477 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Bayçınar Medical Publishing |
record_format | MEDLINE/PubMed |
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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