{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T21:09:40Z","timestamp":1790456980394,"version":"4.1.0"},"reference-count":56,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2018,12,20]],"date-time":"2018-12-20T00:00:00Z","timestamp":1545264000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Institut de Recherche Exp\u00e9rimentale et Clinique"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J of Med Genetics Pt A"],"published-print":{"date-parts":[[2019,3]]},"abstract":"<jats:p>As exertional inspiratory dyspnea is a common disabling complaint in hypermobile Ehlers\u2013Danlos syndrome (hEDS) often also known as joint hypermobility syndrome (JHS), we investigated inspiratory muscle (IM) strength in patients with hEDS, and we assessed the effects of IM training (IMT) on IM strength, lung function, and exercise capacity. A prospective evaluation of IM strength followed by a randomized controlled trial of IMT was performed in women with hEDS. Sniff nasal inspiratory pressure (SNIP) was used to routinely measure IM strength and IMT was carried out using a pressure threshold device. IM strength (main outcome), cardiopulmonary function, exercise capacity, and emotional distress of both the treated and control groups were evaluated at the start and at the end of the 6\u2010week training period. IM strength was reduced (&lt;80% of predicted) in 77% of patients (80\/104). Lung function was normal, although 24% of patients had a higher forced expiratory vital capacity (FVC) than normal and 12% of patients had a higher total lung capacity (TLC) than normal. Both the IMT and control groups (<jats:italic>n<\/jats:italic>\u2009=\u200920) had similar baseline characteristics. Significant changes were noted only in the IMT group after IMT. At the end of the program, IMT improved SNIP (20%) (before: 41\u2009\u00b1\u200917\u2009cm H<jats:sub>2<\/jats:sub>O [28, 53] vs. after: 49\u2009\u00b1\u200918\u2009cm H<jats:sub>2<\/jats:sub>O [34;65]), six\u2010minute walking distance (6MWD) (60\u2009m) (455\u2009\u00b1\u2009107\u2009m [379,532] vs. 515\u2009\u00b1\u2009127\u2009m [408, 621]), and forced expiratory volume in one second (FEV1) (285\u2009mL) (94\u2009\u00b1\u200914% pred [84,104] vs. 103\u2009\u00b1\u200911% pred [94, 112]). IM strength is significantly reduced in patients with hEDS. IMT improved IM strength, lung function, and exercise capacity. Our findings suggest that IMT should be added to usual care.<\/jats:p>","DOI":"10.1002\/ajmg.a.61016","type":"journal-article","created":{"date-parts":[[2018,12,20]],"date-time":"2018-12-20T09:29:09Z","timestamp":1545298149000},"page":"356-364","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":27,"title":["Inspiratory muscle strength training improves lung function in patients with the hypermobile Ehlers\u2013Danlos syndrome: A randomized controlled trial"],"prefix":"10.1002","volume":"179","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7674-1150","authenticated-orcid":false,"given":"Gregory","family":"Reychler","sequence":"first","affiliation":[{"name":"Institut de Recherche Exp\u00e9rimentale et Clinique (IREC), P\u00f4le de Pneumologie, ORL &amp; Dermatologie Universit\u00e9 Catholique de Louvain  Brussels Belgium"},{"name":"Service de Pneumologie Cliniques universitaires Saint\u2010Luc  Brussels Belgium"},{"name":"Service de M\u00e9decine Physique et R\u00e9adaptation Cliniques universitaires Saint\u2010Luc  Brussels Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppe","family":"Liistro","sequence":"additional","affiliation":[{"name":"Institut de Recherche Exp\u00e9rimentale et Clinique (IREC), P\u00f4le de Pneumologie, ORL &amp; Dermatologie Universit\u00e9 Catholique de Louvain  Brussels Belgium"},{"name":"Service de Pneumologie Cliniques universitaires Saint\u2010Luc  Brussels Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G\u00e9rald E.","family":"Pi\u00e9rard","sequence":"additional","affiliation":[{"name":"Laboratory of Skin Bioengineering and Imaging, Department of Dermatopathology University of Li\u00e8ge, University Hospital of Li\u00e8ge  Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Trinh","family":"Hermanns\u2010L\u00ea","sequence":"additional","affiliation":[{"name":"Laboratory of Skin Bioengineering and Imaging, Department of Dermatopathology University of Li\u00e8ge, University Hospital of Li\u00e8ge  Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Manicourt","sequence":"additional","affiliation":[{"name":"Laboratory of Human Molecular Genetics (GEHU), de Duve Institute (DDUV) Universit\u00e9 catholique de Louvain (UCLouvain)  Belgium"},{"name":"Department of Rheumatology University Hospital St Luc  Brussels Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,12,20]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.166.4.518"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1136\/thx.40.4.300"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/ajcn\/36.5.936"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00520-015-2825-3"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/crj.12905"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(19980428)77:1<31::AID-AJMG8>3.0.CO;2-O"},{"key":"e_1_2_9_8_1","first-page":"444","article-title":"Muscle formation in Ehlers\u2010Danlos syndrome","volume":"62","author":"Bilkey W. J.","year":"1981","journal-title":"Archives of Physical Medicine and Rehabilitation"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2011.05.001"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.21557"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.matbio.2013.07.005"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00194909"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.33231"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.33585"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1097\/MOP.0000000000000541"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.c.31539"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1186\/s40064-015-1089-1"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.spinee.2007.01.008"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.150.1.8025735"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00408-014-9649-7"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1081\/COPD-200053377"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.158.5.9710086"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rdc.2013.03.003"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.06.00007906"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0003-9993(00)90105-9"},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.7861\/clinmedicine.13-6-s50"},{"key":"e_1_2_9_27_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.10225"},{"key":"e_1_2_9_28_1","doi-asserted-by":"publisher","DOI":"10.1152\/japplphysiol.01105.2006"},{"key":"e_1_2_9_29_1","doi-asserted-by":"publisher","DOI":"10.1155\/2012\/878107"},{"key":"e_1_2_9_30_1","doi-asserted-by":"publisher","DOI":"10.1186\/1479-5868-5-30"},{"key":"e_1_2_9_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.apmr.2010.06.010"},{"key":"e_1_2_9_32_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00150314"},{"key":"e_1_2_9_33_1","doi-asserted-by":"publisher","DOI":"10.1152\/japplphysiol.00723.2009"},{"key":"e_1_2_9_34_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/37.7.803"},{"key":"e_1_2_9_35_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjmed.2017.02.013"},{"key":"e_1_2_9_36_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40279-017-0747-6"},{"key":"e_1_2_9_37_1","doi-asserted-by":"publisher","DOI":"10.4187\/respcare.02415"},{"key":"e_1_2_9_38_1","doi-asserted-by":"publisher","DOI":"10.1186\/1471-2474-7-82"},{"key":"e_1_2_9_39_1","doi-asserted-by":"publisher","DOI":"10.2147\/COPD.S54925"},{"key":"e_1_2_9_40_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.c.31552"},{"key":"e_1_2_9_41_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.05.00034805"},{"key":"e_1_2_9_42_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2010.03.001"},{"key":"e_1_2_9_43_1","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2006.062224"},{"key":"e_1_2_9_44_1","doi-asserted-by":"publisher","DOI":"10.3109\/09638288.2015.1036171"},{"key":"e_1_2_9_45_1","doi-asserted-by":"publisher","DOI":"10.2147\/COPD.S23784"},{"key":"e_1_2_9_46_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00140507"},{"key":"e_1_2_9_47_1","doi-asserted-by":"publisher","DOI":"10.3109\/09638288.2015.1031286"},{"key":"e_1_2_9_48_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.21726"},{"key":"e_1_2_9_49_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jamda.2016.08.002"},{"key":"e_1_2_9_50_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2005.02.035"},{"key":"e_1_2_9_51_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.162.4.9910034"},{"key":"e_1_2_9_52_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00392-015-0850-3"},{"key":"e_1_2_9_53_1","doi-asserted-by":"publisher","DOI":"10.1136\/thx.50.4.371"},{"key":"e_1_2_9_54_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2007.04.004"},{"key":"e_1_2_9_55_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.physio.2010.06.001"},{"key":"e_1_2_9_56_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.semarthrit.2009.08.003"},{"key":"e_1_2_9_57_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0080321"}],"container-title":["American Journal of Medical Genetics Part A"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fajmg.a.61016","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajmg.a.61016","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/ajmg.a.61016","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajmg.a.61016","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,5]],"date-time":"2023-09-05T20:41:39Z","timestamp":1693946499000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajmg.a.61016"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,12,20]]},"references-count":56,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2019,3]]}},"alternative-id":["10.1002\/ajmg.a.61016"],"URL":"https:\/\/doi.org\/10.1002\/ajmg.a.61016","archive":["Portico"],"relation":{},"ISSN":["1552-4825","1552-4833"],"issn-type":[{"value":"1552-4825","type":"print"},{"value":"1552-4833","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,12,20]]},"assertion":[{"value":"2018-06-26","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2018-11-20","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2018-12-20","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}