Inspiratory muscle strength training improves lung function in patients with the hypermobile Ehlers–Danlos syndrome: A randomized controlled trial
As exertional inspiratory dyspnea is a common disabling complaint in hypermobile Ehlers–Danlos 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 streng...
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| Veröffentlicht in: | American journal of medical genetics. Part A 2019-03, Vol.179 (3), p.356-364 |
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| creator | Reychler, Gregory Liistro, Giuseppe Piérard, Gérald E. Hermanns‐Lê, Trinh Manicourt, Daniel |
| description | As exertional inspiratory dyspnea is a common disabling complaint in hypermobile Ehlers–Danlos 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‐week training period. IM strength was reduced ( |
| doi_str_mv | 10.1002/ajmg.a.61016 |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_2159320551</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>2178843478</sourcerecordid><originalsourceid>FETCH-LOGICAL-c3646-dd49c5c2569bc90f1a0e38fb646f8e4e4c8db534b880d158b354d990f2556c783</originalsourceid><addsrcrecordid>eNp9kU9vFCEYh4nR2Fq9eTYkXjy4Kwwwy3jb1LbW1HjRM2GA2WXDnxGYNuPJ76CfsJ9Etlt78ODpfQkPT96XHwAvMVpihJp3cuc3S7lsMcLtI3CMGWsWlBPy-KFv2BF4lvMOIYLYqn0KjmptO4aaY_D7MuTRJllimqGfsnIG5pJM2JQtLEnaYMMGWj-meG0ydFM9DVNQxcYAbYCjLNaEkuGN3T_YGridR5N87G01nW2dSfn2568PMriYYZ6DTtGb93ANkww6evvDaKhiKCk6V9uSrHTPwZNBumxe3NcT8O387Ovpx8XVl4vL0_XVQpGWtgutaaeYauouverQgCUyhA99vRu4oYYqrntGaM850pjxnjCquwo2jLVqxckJeHPw1u2-TyYX4W1WxjkZTJyyaDDrSIMYwxV9_Q-6i1MKdbpKrTinhN4J3x4olWLOyQxiTNbLNAuMxD4tsU9LSHGXVsVf3Uun3hv9AP-NpwL0ANzU35z_KxPrT58v1gfvH0SopRI</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>2178843478</pqid></control><display><type>article</type><title>Inspiratory muscle strength training improves lung function in patients with the hypermobile Ehlers–Danlos syndrome: A randomized controlled trial</title><source>MEDLINE</source><source>Wiley Online Library Journals Frontfile Complete</source><creator>Reychler, Gregory ; Liistro, Giuseppe ; Piérard, Gérald E. ; Hermanns‐Lê, Trinh ; Manicourt, Daniel</creator><creatorcontrib>Reychler, Gregory ; Liistro, Giuseppe ; Piérard, Gérald E. ; Hermanns‐Lê, Trinh ; Manicourt, Daniel</creatorcontrib><description>As exertional inspiratory dyspnea is a common disabling complaint in hypermobile Ehlers–Danlos 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‐week training period. IM strength was reduced (<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 (n = 20) 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 ± 17 cm H2O [28, 53] vs. after: 49 ± 18 cm H2O [34;65]), six‐minute walking distance (6MWD) (60 m) (455 ± 107 m [379,532] vs. 515 ± 127 m [408, 621]), and forced expiratory volume in one second (FEV1) (285 mL) (94 ± 14% pred [84,104] vs. 103 ± 11% 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.
Key messages
What is the key question?
Are inspiratory muscle strength and lung function normal in Ehlers–Danlos syndrome hypermobility type (EDS‐HT) patients? Can inspiratory muscle training be a potential therapy to improve inspiratory muscle strength, lung function, functional exercise performance, anxiety, and depression?
What is the bottom line?
IM strength was significantly reduced in patients with hEDS, but it was improved by inspiratory muscle training similarly to lung function and exercise capacity.
Why read on?
This is the first description of inspiratory muscle strength and lung function in hEDS and the first trial investigating the effect of inspiratory muscle training in hEDS.</description><identifier>ISSN: 1552-4825</identifier><identifier>EISSN: 1552-4833</identifier><identifier>DOI: 10.1002/ajmg.a.61016</identifier><identifier>PMID: 30569502</identifier><language>eng</language><publisher>Hoboken, USA: John Wiley & Sons, Inc</publisher><subject>Adult ; Anxiety ; Clinical trials ; connective tissue disease associated lung disease ; Dyspnea ; Ehlers-Danlos syndrome ; Ehlers-Danlos Syndrome - diagnosis ; Ehlers-Danlos Syndrome - physiopathology ; Ehlers-Danlos Syndrome - therapy ; Exercise ; Female ; Humans ; Lung - physiopathology ; lung physiology ; Lungs ; Male ; Mental depression ; Middle Aged ; Muscle Strength ; Physical Conditioning, Human - methods ; Pressure ; Resistance Training - methods ; Respiration ; Respiratory function ; Respiratory Function Tests ; Spirometry ; Treatment Outcome ; Walking</subject><ispartof>American journal of medical genetics. Part A, 2019-03, Vol.179 (3), p.356-364</ispartof><rights>2018 Wiley Periodicals, Inc.</rights><rights>2019 Wiley Periodicals, Inc.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c3646-dd49c5c2569bc90f1a0e38fb646f8e4e4c8db534b880d158b354d990f2556c783</citedby><cites>FETCH-LOGICAL-c3646-dd49c5c2569bc90f1a0e38fb646f8e4e4c8db534b880d158b354d990f2556c783</cites><orcidid>0000-0002-7674-1150</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1002%2Fajmg.a.61016$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1002%2Fajmg.a.61016$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,776,780,1411,27901,27902,45550,45551</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/30569502$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Reychler, Gregory</creatorcontrib><creatorcontrib>Liistro, Giuseppe</creatorcontrib><creatorcontrib>Piérard, Gérald E.</creatorcontrib><creatorcontrib>Hermanns‐Lê, Trinh</creatorcontrib><creatorcontrib>Manicourt, Daniel</creatorcontrib><title>Inspiratory muscle strength training improves lung function in patients with the hypermobile Ehlers–Danlos syndrome: A randomized controlled trial</title><title>American journal of medical genetics. Part A</title><addtitle>Am J Med Genet A</addtitle><description>As exertional inspiratory dyspnea is a common disabling complaint in hypermobile Ehlers–Danlos 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‐week training period. IM strength was reduced (<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 (n = 20) 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 ± 17 cm H2O [28, 53] vs. after: 49 ± 18 cm H2O [34;65]), six‐minute walking distance (6MWD) (60 m) (455 ± 107 m [379,532] vs. 515 ± 127 m [408, 621]), and forced expiratory volume in one second (FEV1) (285 mL) (94 ± 14% pred [84,104] vs. 103 ± 11% 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.
Key messages
What is the key question?
Are inspiratory muscle strength and lung function normal in Ehlers–Danlos syndrome hypermobility type (EDS‐HT) patients? Can inspiratory muscle training be a potential therapy to improve inspiratory muscle strength, lung function, functional exercise performance, anxiety, and depression?
What is the bottom line?
IM strength was significantly reduced in patients with hEDS, but it was improved by inspiratory muscle training similarly to lung function and exercise capacity.
Why read on?
This is the first description of inspiratory muscle strength and lung function in hEDS and the first trial investigating the effect of inspiratory muscle training in hEDS.</description><subject>Adult</subject><subject>Anxiety</subject><subject>Clinical trials</subject><subject>connective tissue disease associated lung disease</subject><subject>Dyspnea</subject><subject>Ehlers-Danlos syndrome</subject><subject>Ehlers-Danlos Syndrome - diagnosis</subject><subject>Ehlers-Danlos Syndrome - physiopathology</subject><subject>Ehlers-Danlos Syndrome - therapy</subject><subject>Exercise</subject><subject>Female</subject><subject>Humans</subject><subject>Lung - physiopathology</subject><subject>lung physiology</subject><subject>Lungs</subject><subject>Male</subject><subject>Mental depression</subject><subject>Middle Aged</subject><subject>Muscle Strength</subject><subject>Physical Conditioning, Human - methods</subject><subject>Pressure</subject><subject>Resistance Training - methods</subject><subject>Respiration</subject><subject>Respiratory function</subject><subject>Respiratory Function Tests</subject><subject>Spirometry</subject><subject>Treatment Outcome</subject><subject>Walking</subject><issn>1552-4825</issn><issn>1552-4833</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2019</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kU9vFCEYh4nR2Fq9eTYkXjy4Kwwwy3jb1LbW1HjRM2GA2WXDnxGYNuPJ76CfsJ9Etlt78ODpfQkPT96XHwAvMVpihJp3cuc3S7lsMcLtI3CMGWsWlBPy-KFv2BF4lvMOIYLYqn0KjmptO4aaY_D7MuTRJllimqGfsnIG5pJM2JQtLEnaYMMGWj-meG0ydFM9DVNQxcYAbYCjLNaEkuGN3T_YGridR5N87G01nW2dSfn2568PMriYYZ6DTtGb93ANkww6evvDaKhiKCk6V9uSrHTPwZNBumxe3NcT8O387Ovpx8XVl4vL0_XVQpGWtgutaaeYauouverQgCUyhA99vRu4oYYqrntGaM850pjxnjCquwo2jLVqxckJeHPw1u2-TyYX4W1WxjkZTJyyaDDrSIMYwxV9_Q-6i1MKdbpKrTinhN4J3x4olWLOyQxiTNbLNAuMxD4tsU9LSHGXVsVf3Uun3hv9AP-NpwL0ANzU35z_KxPrT58v1gfvH0SopRI</recordid><startdate>201903</startdate><enddate>201903</enddate><creator>Reychler, Gregory</creator><creator>Liistro, Giuseppe</creator><creator>Piérard, Gérald E.</creator><creator>Hermanns‐Lê, Trinh</creator><creator>Manicourt, Daniel</creator><general>John Wiley & Sons, Inc</general><general>Wiley Subscription Services, Inc</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7QP</scope><scope>7TK</scope><scope>8FD</scope><scope>FR3</scope><scope>K9.</scope><scope>P64</scope><scope>RC3</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0002-7674-1150</orcidid></search><sort><creationdate>201903</creationdate><title>Inspiratory muscle strength training improves lung function in patients with the hypermobile Ehlers–Danlos syndrome: A randomized controlled trial</title><author>Reychler, Gregory ; Liistro, Giuseppe ; Piérard, Gérald E. ; Hermanns‐Lê, Trinh ; Manicourt, Daniel</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3646-dd49c5c2569bc90f1a0e38fb646f8e4e4c8db534b880d158b354d990f2556c783</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2019</creationdate><topic>Adult</topic><topic>Anxiety</topic><topic>Clinical trials</topic><topic>connective tissue disease associated lung disease</topic><topic>Dyspnea</topic><topic>Ehlers-Danlos syndrome</topic><topic>Ehlers-Danlos Syndrome - diagnosis</topic><topic>Ehlers-Danlos Syndrome - physiopathology</topic><topic>Ehlers-Danlos Syndrome - therapy</topic><topic>Exercise</topic><topic>Female</topic><topic>Humans</topic><topic>Lung - physiopathology</topic><topic>lung physiology</topic><topic>Lungs</topic><topic>Male</topic><topic>Mental depression</topic><topic>Middle Aged</topic><topic>Muscle Strength</topic><topic>Physical Conditioning, Human - methods</topic><topic>Pressure</topic><topic>Resistance Training - methods</topic><topic>Respiration</topic><topic>Respiratory function</topic><topic>Respiratory Function Tests</topic><topic>Spirometry</topic><topic>Treatment Outcome</topic><topic>Walking</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Reychler, Gregory</creatorcontrib><creatorcontrib>Liistro, Giuseppe</creatorcontrib><creatorcontrib>Piérard, Gérald E.</creatorcontrib><creatorcontrib>Hermanns‐Lê, Trinh</creatorcontrib><creatorcontrib>Manicourt, Daniel</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Calcium & Calcified Tissue Abstracts</collection><collection>Neurosciences Abstracts</collection><collection>Technology Research Database</collection><collection>Engineering Research Database</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>Genetics Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>American journal of medical genetics. Part A</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Reychler, Gregory</au><au>Liistro, Giuseppe</au><au>Piérard, Gérald E.</au><au>Hermanns‐Lê, Trinh</au><au>Manicourt, Daniel</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Inspiratory muscle strength training improves lung function in patients with the hypermobile Ehlers–Danlos syndrome: A randomized controlled trial</atitle><jtitle>American journal of medical genetics. Part A</jtitle><addtitle>Am J Med Genet A</addtitle><date>2019-03</date><risdate>2019</risdate><volume>179</volume><issue>3</issue><spage>356</spage><epage>364</epage><pages>356-364</pages><issn>1552-4825</issn><eissn>1552-4833</eissn><abstract>As exertional inspiratory dyspnea is a common disabling complaint in hypermobile Ehlers–Danlos 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‐week training period. IM strength was reduced (<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 (n = 20) 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 ± 17 cm H2O [28, 53] vs. after: 49 ± 18 cm H2O [34;65]), six‐minute walking distance (6MWD) (60 m) (455 ± 107 m [379,532] vs. 515 ± 127 m [408, 621]), and forced expiratory volume in one second (FEV1) (285 mL) (94 ± 14% pred [84,104] vs. 103 ± 11% 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.
Key messages
What is the key question?
Are inspiratory muscle strength and lung function normal in Ehlers–Danlos syndrome hypermobility type (EDS‐HT) patients? Can inspiratory muscle training be a potential therapy to improve inspiratory muscle strength, lung function, functional exercise performance, anxiety, and depression?
What is the bottom line?
IM strength was significantly reduced in patients with hEDS, but it was improved by inspiratory muscle training similarly to lung function and exercise capacity.
Why read on?
This is the first description of inspiratory muscle strength and lung function in hEDS and the first trial investigating the effect of inspiratory muscle training in hEDS.</abstract><cop>Hoboken, USA</cop><pub>John Wiley & Sons, Inc</pub><pmid>30569502</pmid><doi>10.1002/ajmg.a.61016</doi><tpages>9</tpages><orcidid>https://orcid.org/0000-0002-7674-1150</orcidid></addata></record> |
| fulltext | fulltext |
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| ispartof | American journal of medical genetics. Part A, 2019-03, Vol.179 (3), p.356-364 |
| issn | 1552-4825 1552-4833 |
| language | eng |
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| source | MEDLINE; Wiley Online Library Journals Frontfile Complete |
| subjects | Adult Anxiety Clinical trials connective tissue disease associated lung disease Dyspnea Ehlers-Danlos syndrome Ehlers-Danlos Syndrome - diagnosis Ehlers-Danlos Syndrome - physiopathology Ehlers-Danlos Syndrome - therapy Exercise Female Humans Lung - physiopathology lung physiology Lungs Male Mental depression Middle Aged Muscle Strength Physical Conditioning, Human - methods Pressure Resistance Training - methods Respiration Respiratory function Respiratory Function Tests Spirometry Treatment Outcome Walking |
| title | Inspiratory muscle strength training improves lung function in patients with the hypermobile Ehlers–Danlos syndrome: A randomized controlled trial |
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