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Case report of endoprosthesis -Y implantation in severe respiratory failure in the MPSII patient; comparison with literature data
BACKGROUND: The tracheobronchomalacia is a life-threatening complication of mucopolysaccharidosis (MPS) without known effective, optimal treatment. The severe expiratory collapse of the trachea and bronchi is one of causes of the high rate of deaths in the course of airway impairment in MPSII patien...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7171830/ https://www.ncbi.nlm.nih.gov/pubmed/32312280 http://dx.doi.org/10.1186/s12890-020-1143-9 |
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author | Gocyk, Wojciech Warmus, Janusz Olechnowicz, Henryk Bik-Multanowski, Miroslaw Pawlinski, Lukasz Kiec-Wilk, Beata |
author_facet | Gocyk, Wojciech Warmus, Janusz Olechnowicz, Henryk Bik-Multanowski, Miroslaw Pawlinski, Lukasz Kiec-Wilk, Beata |
author_sort | Gocyk, Wojciech |
collection | PubMed |
description | BACKGROUND: The tracheobronchomalacia is a life-threatening complication of mucopolysaccharidosis (MPS) without known effective, optimal treatment. The severe expiratory collapse of the trachea and bronchi is one of causes of the high rate of deaths in the course of airway impairment in MPSII patients. CASE PRESENTATION: Due to the adynamic tracheobronchomalacia despite of enzymatic treatment (ERT) in our MPSII patient, a life-saving tracheal bifurcated type-Y endoprosthesis (a self-expanding, metal stent for the prosthesis of tracheal and bronchial stenosis) was implanted. In the followed months, the breathing efficiency improved, but then gradual worsening, progression of bronchi occlusion at the stent border resulted in patient’s death. CONCLUSION: The Y-stent implantation appears to be a short-term, life-saving solution without satisfactory long-term effects due to the progress of peripheral bronchomalacia and increased tissue proliferation and granulation, that arises during the illness’ course. |
format | Online Article Text |
id | pubmed-7171830 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-71718302020-04-24 Case report of endoprosthesis -Y implantation in severe respiratory failure in the MPSII patient; comparison with literature data Gocyk, Wojciech Warmus, Janusz Olechnowicz, Henryk Bik-Multanowski, Miroslaw Pawlinski, Lukasz Kiec-Wilk, Beata BMC Pulm Med Case Report BACKGROUND: The tracheobronchomalacia is a life-threatening complication of mucopolysaccharidosis (MPS) without known effective, optimal treatment. The severe expiratory collapse of the trachea and bronchi is one of causes of the high rate of deaths in the course of airway impairment in MPSII patients. CASE PRESENTATION: Due to the adynamic tracheobronchomalacia despite of enzymatic treatment (ERT) in our MPSII patient, a life-saving tracheal bifurcated type-Y endoprosthesis (a self-expanding, metal stent for the prosthesis of tracheal and bronchial stenosis) was implanted. In the followed months, the breathing efficiency improved, but then gradual worsening, progression of bronchi occlusion at the stent border resulted in patient’s death. CONCLUSION: The Y-stent implantation appears to be a short-term, life-saving solution without satisfactory long-term effects due to the progress of peripheral bronchomalacia and increased tissue proliferation and granulation, that arises during the illness’ course. BioMed Central 2020-04-20 /pmc/articles/PMC7171830/ /pubmed/32312280 http://dx.doi.org/10.1186/s12890-020-1143-9 Text en © The Author(s). 2020 Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. 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 in a credit line to the data. |
spellingShingle | Case Report Gocyk, Wojciech Warmus, Janusz Olechnowicz, Henryk Bik-Multanowski, Miroslaw Pawlinski, Lukasz Kiec-Wilk, Beata Case report of endoprosthesis -Y implantation in severe respiratory failure in the MPSII patient; comparison with literature data |
title | Case report of endoprosthesis -Y implantation in severe respiratory failure in the MPSII patient; comparison with literature data |
title_full | Case report of endoprosthesis -Y implantation in severe respiratory failure in the MPSII patient; comparison with literature data |
title_fullStr | Case report of endoprosthesis -Y implantation in severe respiratory failure in the MPSII patient; comparison with literature data |
title_full_unstemmed | Case report of endoprosthesis -Y implantation in severe respiratory failure in the MPSII patient; comparison with literature data |
title_short | Case report of endoprosthesis -Y implantation in severe respiratory failure in the MPSII patient; comparison with literature data |
title_sort | case report of endoprosthesis -y implantation in severe respiratory failure in the mpsii patient; comparison with literature data |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7171830/ https://www.ncbi.nlm.nih.gov/pubmed/32312280 http://dx.doi.org/10.1186/s12890-020-1143-9 |
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