Adrien Hakimi1,2, Cyrille Bergoin3,4, Patrick Mucci5. 1. Univ. Lille, Univ. Artois, Univ. Littoral Côte d'Opale, ULR 7369-URePSSS-Unité de Recherche Pluridisciplinaire Sport Santé Société, F-59000, Lille, France. adrien.hakimi.etu@univ-lille.fr. 2. Clinique de la Mitterie, Lomme, France. adrien.hakimi.etu@univ-lille.fr. 3. Clinique de la Mitterie, Lomme, France. 4. Cabinet de Pneumologie, Tourcoing, France. 5. Univ. Lille, Univ. Artois, Univ. Littoral Côte d'Opale, ULR 7369-URePSSS-Unité de Recherche Pluridisciplinaire Sport Santé Société, F-59000, Lille, France.
Abstract
PURPOSE: Hypermobile Ehlers-Danlos syndrome (hEDS) is a connective tissue disorder with many different symptoms such as pain, fatigue, dysautonomia, or respiratory symptoms. Among the respiratory manifestations described, the most frequent are exertional dyspnea and breathing difficulties. Mechanical ventilatory constraints during exercise could participate in these respiratory manifestations. The objective of this study was to explore the response of pulmonary flow-volume loops to exercise in patients with hEDS and to look for dynamic hyperinflation and expiratory flow limitation during exercise. METHODS: For this purpose, breathing pattern and tidal exercise flow-volume loops were recorded at two workloads (30% and 80% of the peak power output) of a constant load exercise test. RESULTS: Twelve patients were included (11 women, mean age 41 ± 14 years). The results showed a decrease (p = 0.028) in the inspiratory capacity (from 3.12 ± 0.49 L to 2.97 ± 0.52 L), an increase (p = 0.025) in the end-expiratory lung volume (from 0.73 ± 0.68 L to 0.88 ± 0.66 L, i.e., from EELV comprising 17 ± 12% to 21 ± 12% of forced vital capacity) between the two workloads in favor of dynamic hyperinflation, and half of the patients had expiratory flow limitations. CONCLUSION: This exploratory study provides evidence for mechanical ventilatory constraints during exercise in patients with hEDS, which may induce discomfort during exercise and could contribute to the respiratory symptomatology. TRIAL REGISTRATION NUMBER: This study is part of a larger clinical trial (ID: NCT04680793, December 2020).
PURPOSE: Hypermobile Ehlers-Danlos syndrome (hEDS) is a connective tissue disorder with many different symptoms such as pain, fatigue, dysautonomia, or respiratory symptoms. Among the respiratory manifestations described, the most frequent are exertional dyspnea and breathing difficulties. Mechanical ventilatory constraints during exercise could participate in these respiratory manifestations. The objective of this study was to explore the response of pulmonary flow-volume loops to exercise in patients with hEDS and to look for dynamic hyperinflation and expiratory flow limitation during exercise. METHODS: For this purpose, breathing pattern and tidal exercise flow-volume loops were recorded at two workloads (30% and 80% of the peak power output) of a constant load exercise test. RESULTS: Twelve patients were included (11 women, mean age 41 ± 14 years). The results showed a decrease (p = 0.028) in the inspiratory capacity (from 3.12 ± 0.49 L to 2.97 ± 0.52 L), an increase (p = 0.025) in the end-expiratory lung volume (from 0.73 ± 0.68 L to 0.88 ± 0.66 L, i.e., from EELV comprising 17 ± 12% to 21 ± 12% of forced vital capacity) between the two workloads in favor of dynamic hyperinflation, and half of the patients had expiratory flow limitations. CONCLUSION: This exploratory study provides evidence for mechanical ventilatory constraints during exercise in patients with hEDS, which may induce discomfort during exercise and could contribute to the respiratory symptomatology. TRIAL REGISTRATION NUMBER: This study is part of a larger clinical trial (ID: NCT04680793, December 2020).
Authors: Daniel Langer; Casey E Ciavaglia; J Alberto Neder; Katherine A Webb; Denis E O'Donnell Journal: Expert Rev Respir Med Date: 2014-08-27 Impact factor: 3.772
Authors: Marco Castori; Filippo Camerota; Claudia Celletti; Chiara Danese; Valter Santilli; Vincenzo Maria Saraceni; Paola Grammatico Journal: Am J Med Genet A Date: 2010-03 Impact factor: 2.802
Authors: Fransiska Malfait; Clair Francomano; Peter Byers; John Belmont; Britta Berglund; James Black; Lara Bloom; Jessica M Bowen; Angela F Brady; Nigel P Burrows; Marco Castori; Helen Cohen; Marina Colombi; Serwet Demirdas; Julie De Backer; Anne De Paepe; Sylvie Fournel-Gigleux; Michael Frank; Neeti Ghali; Cecilia Giunta; Rodney Grahame; Alan Hakim; Xavier Jeunemaitre; Diana Johnson; Birgit Juul-Kristensen; Ines Kapferer-Seebacher; Hanadi Kazkaz; Tomoki Kosho; Mark E Lavallee; Howard Levy; Roberto Mendoza-Londono; Melanie Pepin; F Michael Pope; Eyal Reinstein; Leema Robert; Marianne Rohrbach; Lynn Sanders; Glenda J Sobey; Tim Van Damme; Anthony Vandersteen; Caroline van Mourik; Nicol Voermans; Nigel Wheeldon; Johannes Zschocke; Brad Tinkle Journal: Am J Med Genet C Semin Med Genet Date: 2017-03 Impact factor: 3.908