Determinants of cough efficiency in Duchenne muscular dystrophy

Summary Objective With the progression of Duchenne muscular dystrophy (DMD) cough becomes inefficient leading to recurrent chest infections. Several factors determine the effectiveness of cough in DMD patients. The aim of this study was to investigate how weakened inspiratory muscles alter operating...

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Veröffentlicht in:Pediatric pulmonology 2014-04, Vol.49 (4), p.357-365
Hauptverfasser: LoMauro, Antonella, Romei, Marianna, D'Angelo, Maria Grazia, Aliverti, Andrea
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container_title Pediatric pulmonology
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creator LoMauro, Antonella
Romei, Marianna
D'Angelo, Maria Grazia
Aliverti, Andrea
description Summary Objective With the progression of Duchenne muscular dystrophy (DMD) cough becomes inefficient leading to recurrent chest infections. Several factors determine the effectiveness of cough in DMD patients. The aim of this study was to investigate how weakened inspiratory muscles alter operating lung and thoraco‐abdominal volumes and whether they contribute to cough efficiency. Patients and Methods Pulmonary function, respiratory muscle strength, and peak cough flow (PCF) were assessed in 36 DMD patients (age 17.0 ± 5.0). Total and compartmental chest wall volumes were measured by Opto‐Electronic Plethysmography in the DMD patients and 15 age‐matched controls during quiet breathing and maximal voluntary cough maneuvers. The DMD population was divided into three groups: PCF  270 L/min (efficient cough), and 160 
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Several factors determine the effectiveness of cough in DMD patients. The aim of this study was to investigate how weakened inspiratory muscles alter operating lung and thoraco‐abdominal volumes and whether they contribute to cough efficiency. Patients and Methods Pulmonary function, respiratory muscle strength, and peak cough flow (PCF) were assessed in 36 DMD patients (age 17.0 ± 5.0). Total and compartmental chest wall volumes were measured by Opto‐Electronic Plethysmography in the DMD patients and 15 age‐matched controls during quiet breathing and maximal voluntary cough maneuvers. The DMD population was divided into three groups: PCF < 160 L/min (inefficient cough), PCF > 270 L/min (efficient cough), and 160 < PCF < 270 L/min (intermediate cough efficiency). Results During the inspiration preceding cough, patients with efficient cough presented normal volume variations whereas patients with intermediate cough efficiency showed low abdominal volume variation (P < 0.01). Patients with inefficient cough were characterized by reduced total (P < 0.05) and compartmental (ribcage: P < 0.01; abdomen: P < 0.001) chest wall volumes during the inspiration preceding cough and reduced abdominal contribution to tidal volume during quiet breathing (ΔVAB (%VT), P < 0.001). ROC analysis revealed that among all spirometric, respiratory muscle strength and chest wall parameters ΔVAB (%VT) was the best discriminator between inefficient and efficient cough. Conclusions Inefficient cough in DMD is associated to reduced operating lung and chest wall volume secondary to weakened inspiratory muscles. Abdominal contribution to tidal volume during spontaneous breathing represents a non‐volitional and noninvasive index able to discriminate efficient and inefficient cough. Pediatr Pulmonol. 2014; 49:357–365. © 2013 Wiley Periodicals, Inc.]]></description><identifier>ISSN: 8755-6863</identifier><identifier>EISSN: 1099-0496</identifier><identifier>DOI: 10.1002/ppul.22836</identifier><identifier>PMID: 23852963</identifier><language>eng</language><publisher>United States: Blackwell Publishing Ltd</publisher><subject>abdomen ; Adolescent ; Adult ; chest wall ; Child ; Cough ; Humans ; Male ; Muscular Dystrophy, Duchenne - physiopathology ; Peak Expiratory Flow Rate ; respiratory muscles ; Respiratory Muscles - physiopathology ; Thoracic Wall - physiopathology ; Tidal Volume ; Young Adult</subject><ispartof>Pediatric pulmonology, 2014-04, Vol.49 (4), p.357-365</ispartof><rights>2013 Wiley Periodicals, Inc.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4626-9bf659be51aad642e7834cddf9d50044537f1e2fc2c6d76e2abc0c4c37d62a693</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1002%2Fppul.22836$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1002%2Fppul.22836$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,780,784,1417,27924,27925,45574,45575</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/23852963$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>LoMauro, Antonella</creatorcontrib><creatorcontrib>Romei, Marianna</creatorcontrib><creatorcontrib>D'Angelo, Maria Grazia</creatorcontrib><creatorcontrib>Aliverti, Andrea</creatorcontrib><title>Determinants of cough efficiency in Duchenne muscular dystrophy</title><title>Pediatric pulmonology</title><addtitle>Pediatr Pulmonol</addtitle><description><![CDATA[Summary Objective With the progression of Duchenne muscular dystrophy (DMD) cough becomes inefficient leading to recurrent chest infections. Several factors determine the effectiveness of cough in DMD patients. The aim of this study was to investigate how weakened inspiratory muscles alter operating lung and thoraco‐abdominal volumes and whether they contribute to cough efficiency. Patients and Methods Pulmonary function, respiratory muscle strength, and peak cough flow (PCF) were assessed in 36 DMD patients (age 17.0 ± 5.0). Total and compartmental chest wall volumes were measured by Opto‐Electronic Plethysmography in the DMD patients and 15 age‐matched controls during quiet breathing and maximal voluntary cough maneuvers. The DMD population was divided into three groups: PCF < 160 L/min (inefficient cough), PCF > 270 L/min (efficient cough), and 160 < PCF < 270 L/min (intermediate cough efficiency). Results During the inspiration preceding cough, patients with efficient cough presented normal volume variations whereas patients with intermediate cough efficiency showed low abdominal volume variation (P < 0.01). Patients with inefficient cough were characterized by reduced total (P < 0.05) and compartmental (ribcage: P < 0.01; abdomen: P < 0.001) chest wall volumes during the inspiration preceding cough and reduced abdominal contribution to tidal volume during quiet breathing (ΔVAB (%VT), P < 0.001). ROC analysis revealed that among all spirometric, respiratory muscle strength and chest wall parameters ΔVAB (%VT) was the best discriminator between inefficient and efficient cough. Conclusions Inefficient cough in DMD is associated to reduced operating lung and chest wall volume secondary to weakened inspiratory muscles. Abdominal contribution to tidal volume during spontaneous breathing represents a non‐volitional and noninvasive index able to discriminate efficient and inefficient cough. Pediatr Pulmonol. 2014; 49:357–365. © 2013 Wiley Periodicals, Inc.]]></description><subject>abdomen</subject><subject>Adolescent</subject><subject>Adult</subject><subject>chest wall</subject><subject>Child</subject><subject>Cough</subject><subject>Humans</subject><subject>Male</subject><subject>Muscular Dystrophy, Duchenne - physiopathology</subject><subject>Peak Expiratory Flow Rate</subject><subject>respiratory muscles</subject><subject>Respiratory Muscles - physiopathology</subject><subject>Thoracic Wall - physiopathology</subject><subject>Tidal Volume</subject><subject>Young Adult</subject><issn>8755-6863</issn><issn>1099-0496</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2014</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpdkMtOwzAQRS0EouWx4QNQJDZsUvx2vEKo5SVVwAIEO8t1JjSQF3EsyN-TtsCC1Yx0zx2NDkJHBE8IxvSsaUIxoTRhcguNCdY6xlzLbTROlBCxTCQboT3v3zAeMk120YiyRFAt2Ridz6CDtswrW3U-qrPI1eF1GUGW5S6HyvVRXkWz4JZQVRCVwbtQ2DZKe9-1dbPsD9BOZgsPhz9zHz1dXT5Ob-L5_fXt9GIeOy6pjPUik0IvQBBrU8kpqIRxl6aZTgXGnAumMgI0c9TJVEmgduGw446pVFIrNdtHp5u7TVt_BPCdKXPvoChsBXXwhgiccMokX6En_9C3OrTV8N2KUkQzkZCBOv6hwqKE1DRtXtq2N79qBoBsgM-8gP4vJ9ispJuVdLOWbh4enubrbejEm07uO_j669j23UjFlDDPd9dG0eEBPKfmhX0DVYqC5Q</recordid><startdate>201404</startdate><enddate>201404</enddate><creator>LoMauro, Antonella</creator><creator>Romei, Marianna</creator><creator>D'Angelo, Maria Grazia</creator><creator>Aliverti, Andrea</creator><general>Blackwell Publishing Ltd</general><general>Wiley Subscription Services, Inc</general><scope>BSCLL</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>K9.</scope><scope>7X8</scope></search><sort><creationdate>201404</creationdate><title>Determinants of cough efficiency in Duchenne muscular dystrophy</title><author>LoMauro, Antonella ; Romei, Marianna ; D'Angelo, Maria Grazia ; Aliverti, Andrea</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4626-9bf659be51aad642e7834cddf9d50044537f1e2fc2c6d76e2abc0c4c37d62a693</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2014</creationdate><topic>abdomen</topic><topic>Adolescent</topic><topic>Adult</topic><topic>chest wall</topic><topic>Child</topic><topic>Cough</topic><topic>Humans</topic><topic>Male</topic><topic>Muscular Dystrophy, Duchenne - physiopathology</topic><topic>Peak Expiratory Flow Rate</topic><topic>respiratory muscles</topic><topic>Respiratory Muscles - physiopathology</topic><topic>Thoracic Wall - physiopathology</topic><topic>Tidal Volume</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>LoMauro, Antonella</creatorcontrib><creatorcontrib>Romei, Marianna</creatorcontrib><creatorcontrib>D'Angelo, Maria Grazia</creatorcontrib><creatorcontrib>Aliverti, Andrea</creatorcontrib><collection>Istex</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>ProQuest Health &amp; Medical Complete (Alumni)</collection><collection>MEDLINE - Academic</collection><jtitle>Pediatric pulmonology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>LoMauro, Antonella</au><au>Romei, Marianna</au><au>D'Angelo, Maria Grazia</au><au>Aliverti, Andrea</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Determinants of cough efficiency in Duchenne muscular dystrophy</atitle><jtitle>Pediatric pulmonology</jtitle><addtitle>Pediatr Pulmonol</addtitle><date>2014-04</date><risdate>2014</risdate><volume>49</volume><issue>4</issue><spage>357</spage><epage>365</epage><pages>357-365</pages><issn>8755-6863</issn><eissn>1099-0496</eissn><abstract><![CDATA[Summary Objective With the progression of Duchenne muscular dystrophy (DMD) cough becomes inefficient leading to recurrent chest infections. Several factors determine the effectiveness of cough in DMD patients. The aim of this study was to investigate how weakened inspiratory muscles alter operating lung and thoraco‐abdominal volumes and whether they contribute to cough efficiency. Patients and Methods Pulmonary function, respiratory muscle strength, and peak cough flow (PCF) were assessed in 36 DMD patients (age 17.0 ± 5.0). Total and compartmental chest wall volumes were measured by Opto‐Electronic Plethysmography in the DMD patients and 15 age‐matched controls during quiet breathing and maximal voluntary cough maneuvers. The DMD population was divided into three groups: PCF < 160 L/min (inefficient cough), PCF > 270 L/min (efficient cough), and 160 < PCF < 270 L/min (intermediate cough efficiency). Results During the inspiration preceding cough, patients with efficient cough presented normal volume variations whereas patients with intermediate cough efficiency showed low abdominal volume variation (P < 0.01). Patients with inefficient cough were characterized by reduced total (P < 0.05) and compartmental (ribcage: P < 0.01; abdomen: P < 0.001) chest wall volumes during the inspiration preceding cough and reduced abdominal contribution to tidal volume during quiet breathing (ΔVAB (%VT), P < 0.001). ROC analysis revealed that among all spirometric, respiratory muscle strength and chest wall parameters ΔVAB (%VT) was the best discriminator between inefficient and efficient cough. Conclusions Inefficient cough in DMD is associated to reduced operating lung and chest wall volume secondary to weakened inspiratory muscles. Abdominal contribution to tidal volume during spontaneous breathing represents a non‐volitional and noninvasive index able to discriminate efficient and inefficient cough. Pediatr Pulmonol. 2014; 49:357–365. © 2013 Wiley Periodicals, Inc.]]></abstract><cop>United States</cop><pub>Blackwell Publishing Ltd</pub><pmid>23852963</pmid><doi>10.1002/ppul.22836</doi><tpages>9</tpages><oa>free_for_read</oa></addata></record>
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subjects abdomen
Adolescent
Adult
chest wall
Child
Cough
Humans
Male
Muscular Dystrophy, Duchenne - physiopathology
Peak Expiratory Flow Rate
respiratory muscles
Respiratory Muscles - physiopathology
Thoracic Wall - physiopathology
Tidal Volume
Young Adult
title Determinants of cough efficiency in Duchenne muscular dystrophy
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