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Lung Function Changes are More Common in Marfan Patients Who Need Major Thoracic Surgery

INTRODUCTION: Marfan syndrome is a genetic disorder affecting the connective tissue. Changes in lung tissue might influence respiratory function; however, a detailed respiratory functional assessment according to the need for major thoracic surgery is missing. METHODS: Comprehensive pulmonary examin...

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Autores principales: Kolonics-Farkas, Abigel M., Agg, Bence, Benke, Kalman, Odler, Balazs, Bohacs, Aniko, Kovats, Zsuzsanna, Szabolcs, Zoltan, Müller, Veronika
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer US 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6647216/
https://www.ncbi.nlm.nih.gov/pubmed/31089858
http://dx.doi.org/10.1007/s00408-019-00236-1
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author Kolonics-Farkas, Abigel M.
Agg, Bence
Benke, Kalman
Odler, Balazs
Bohacs, Aniko
Kovats, Zsuzsanna
Szabolcs, Zoltan
Müller, Veronika
author_facet Kolonics-Farkas, Abigel M.
Agg, Bence
Benke, Kalman
Odler, Balazs
Bohacs, Aniko
Kovats, Zsuzsanna
Szabolcs, Zoltan
Müller, Veronika
author_sort Kolonics-Farkas, Abigel M.
collection PubMed
description INTRODUCTION: Marfan syndrome is a genetic disorder affecting the connective tissue. Changes in lung tissue might influence respiratory function; however, a detailed respiratory functional assessment according to the need for major thoracic surgery is missing. METHODS: Comprehensive pulmonary examinations were performed in 55 Marfan patients including respiratory symptoms, lung function (LF) testing using European Coal and Steel Community (ECSC) reference values, TL(CO) and quality of life measurements. Groups included patients who did not need surgery (Mf, n = 32) and those who underwent major thoracic surgery (Mf(op), n = 23). RESULTS: Respiratory symptoms affected 20% of patients. Scoliosis was significantly more frequent in the Mf(op) group. LF demonstrated in all Marfan patients a tendency towards airway obstruction (FEV1/FVC = 0.77 ± 0.10), more prominent in Mf(op) patients (0.74 ± 0.08 vs. Mf: 0.80 ± 0.11; p = 0.03). Correction of LF values using a standing height modification by arm span (H(corrected)) revealed additional changes in FVC and FEV1. TL(CO) and quality of life did not differ between groups. CONCLUSIONS: Marfan syndrome is associated with airway obstruction, especially in patients who have undergone major thoracic surgery, indicative of more severe connective tissue malfunction. The use of arm span for height correction is suitable to evaluate LF changes in this special patient group including patients with significant scoliosis.
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spelling pubmed-66472162019-08-06 Lung Function Changes are More Common in Marfan Patients Who Need Major Thoracic Surgery Kolonics-Farkas, Abigel M. Agg, Bence Benke, Kalman Odler, Balazs Bohacs, Aniko Kovats, Zsuzsanna Szabolcs, Zoltan Müller, Veronika Lung Respiratory Physiology INTRODUCTION: Marfan syndrome is a genetic disorder affecting the connective tissue. Changes in lung tissue might influence respiratory function; however, a detailed respiratory functional assessment according to the need for major thoracic surgery is missing. METHODS: Comprehensive pulmonary examinations were performed in 55 Marfan patients including respiratory symptoms, lung function (LF) testing using European Coal and Steel Community (ECSC) reference values, TL(CO) and quality of life measurements. Groups included patients who did not need surgery (Mf, n = 32) and those who underwent major thoracic surgery (Mf(op), n = 23). RESULTS: Respiratory symptoms affected 20% of patients. Scoliosis was significantly more frequent in the Mf(op) group. LF demonstrated in all Marfan patients a tendency towards airway obstruction (FEV1/FVC = 0.77 ± 0.10), more prominent in Mf(op) patients (0.74 ± 0.08 vs. Mf: 0.80 ± 0.11; p = 0.03). Correction of LF values using a standing height modification by arm span (H(corrected)) revealed additional changes in FVC and FEV1. TL(CO) and quality of life did not differ between groups. CONCLUSIONS: Marfan syndrome is associated with airway obstruction, especially in patients who have undergone major thoracic surgery, indicative of more severe connective tissue malfunction. The use of arm span for height correction is suitable to evaluate LF changes in this special patient group including patients with significant scoliosis. Springer US 2019-05-14 2019 /pmc/articles/PMC6647216/ /pubmed/31089858 http://dx.doi.org/10.1007/s00408-019-00236-1 Text en © The Author(s) 2019 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.
spellingShingle Respiratory Physiology
Kolonics-Farkas, Abigel M.
Agg, Bence
Benke, Kalman
Odler, Balazs
Bohacs, Aniko
Kovats, Zsuzsanna
Szabolcs, Zoltan
Müller, Veronika
Lung Function Changes are More Common in Marfan Patients Who Need Major Thoracic Surgery
title Lung Function Changes are More Common in Marfan Patients Who Need Major Thoracic Surgery
title_full Lung Function Changes are More Common in Marfan Patients Who Need Major Thoracic Surgery
title_fullStr Lung Function Changes are More Common in Marfan Patients Who Need Major Thoracic Surgery
title_full_unstemmed Lung Function Changes are More Common in Marfan Patients Who Need Major Thoracic Surgery
title_short Lung Function Changes are More Common in Marfan Patients Who Need Major Thoracic Surgery
title_sort lung function changes are more common in marfan patients who need major thoracic surgery
topic Respiratory Physiology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6647216/
https://www.ncbi.nlm.nih.gov/pubmed/31089858
http://dx.doi.org/10.1007/s00408-019-00236-1
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