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      Assisted Cough and Pulmonary Compliance in Patients with Duchenne Muscular Dystrophy

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          Abstract

          The aim of this study was to investigate the factors affecting cough ability, and to compare the assisted cough methods in patients with Duchenne muscular dystrophy (DMD). A total seventy-one male patients with DMD were included in the study. The vital capacity (VC) and maximum insufflation capacity (MIC) were measured. The unassisted peak cough flow (UPCF) and three different techniques of assisted peak cough flow were evaluated. UPCF measurements were possible for all 71 subjects. But when performing the three different assisted cough techniques, peak cough flows (PCFs) could be obtained from only 51 subjects. The mean value of MICs (1801 ± 780 cc) was higher than that of VCs (1502 ± 765 cc) ( p < 0.01). All three assisted cough methods showed a significantly higher value than the unassisted method (F=80.92, p < 0.01). The manual assisted PCF under MIC (MPCFmic) significantly exceeded those produced by manual assisted PCF (MPCF) or PCF under MIC (PCFmic). The positive correlation between the MIC, VC difference (MIC-VC), and the difference between PCFmic and UPCF (PCFmic-UPCF) was seen (r=0.572, p < 0.01). The preservation of pulmonary compliance is important for the development of an effective cough as well as assisting the compression and expulsive phases. Thus, the clinical importance of the inspiratory phase and pulmonary compliance in assisting a cough should be emphasized.

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          Most cited references23

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          Prevention of pulmonary morbidity for patients with neuromuscular disease.

          To evaluate the effects of a respiratory muscle aid protocol on hospitalization rates for respiratory complications of neuromuscular disease. A retrospective cohort study. A home protocol was developed in which oxyhemoglobin desaturation was prevented or reversed by the use of noninvasive intermittent positive-pressure ventilation and manually and mechanically assisted coughing as needed. The patients who had more than one episode of respiratory failure before having access to the protocol were considered to have had preprotocol periods (group 1). Other patients were given access to the protocol when their assisted peak cough flows decreased to < 270 L/min before any episodes of respiratory distress (group 2). The number of hospitalizations and days hospitalized were compared longitudinally for preprotocol and protocol access periods (group 1). In addition, avoided hospitalizations were identified as "episodes" of need for continuous ventilatory support and desaturations reversed by assisted coughing that were managed at home. Data were segregated by access to protocol and by extent of baseline ventilator use. Of the 47 group 1 patients with preprotocol periods who have subsequently had episodes, 10 had episodes before requiring ongoing ventilator use. They had 1.06 +/- 0.84 preprotocol hospitalizations per year per patient and 20.76 +/- 36.01 hospitalization days per year per patient over 3.42 +/- 3.36 years per patient vs 0.03 +/- 0.11 hospitalizations per year per patient and 0.06 +/- 0.20 hospitalization days per year per patient with protocol use over 1.94 +/- 0.74 years per patient. Of these 47 group 1 patients, 33 eventually required part-time ventilatory aid and, using the protocol as needed, had 0.08 +/- 0.17 hospitalizations per year per patient and 1.43 +/- 3.71 hospitalization days per year per patient over 3.91 +/- 3.50 years per patient, as opposed to 1.40 +/- 1.96 hospitalizations per year per patient and 20.14 +/- 41.15 hospitalization days per year per patient preprotocol and preventilator use over 5.89 +/- 6.89 years per patient. Twelve patients in group 1 eventually required continuous noninvasive ventilation and, using the protocol as needed, had 0.07 +/- 0.14 hospitalizations per year per patient and 0.39 +/- 0.73 hospitalization days per year per patient over 5.35 +/- 5.10 years per patient by comparison with 0.97 +/- 0.74 hospitalizations per year per patient and 10.39 +/- 8.66 hospitalization days per year per patient over 2.18 +/- 1.91 years per patient preprotocol and preventilator use. For the 94 patients overall when having access to the protocol, 1.02 +/- 0.99 hospitalizations per year per patient were avoided by 14 patients before requiring ongoing ventilator use over 4.82 +/- 1.61 years, 0.99 +/- 1.12 hospitalizations per year per patient were avoided by 73 part-time ventilator users over 3.21 +/- 3.15 years, and 0.80 +/- 0.85 hospitalizations per year per patient were avoided by 31 full-time ventilator users over 4.78 +/- 4.88 years. All preprotocol and protocol rate comparisons were statistically significant at p < 0.004. Patients have significantly fewer hospitalizations per year and days per year when using the protocol as needed than without the protocol. The use of inspiratory and expiratory aids can significantly decrease hospitalization rates for respiratory complications of neuromuscular disease.
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            Cough augmentation with mechanical insufflation/exsufflation in patients with neuromuscular weakness.

            Adults and children with neuromuscular disease exhibit weak cough and are susceptible to recurrent chest infections, a major cause of morbidity and mortality. Mechanical insufflation/exsufflation may improve cough efficacy by increasing peak cough flow. It was hypothesised that mechanical insufflation/exsufflation would produce a greater increase in peak cough flow than other modes of cough augmentation. The acceptability of these interventions was also compared. Twenty-two patients aged 10-56 yrs (median 21 yrs) with neuromuscular disease and 19 age-matched controls were studied. Spirometry was performed and respiratory muscle strength measured. Peak cough flow was recorded during maximal unassisted coughs, followed in random order by coughs assisted by physiotherapy, noninvasive ventilation, insufflation and exsufflation, and exsufflation alone. Subjects rated strength of cough, distress and comfort on a visual analogue scale. In the neuromuscular disease group, mean +/- SD forced expiratory volume in one second was 0.8 +/- 0.6 L x s(-1), forced vital capacity 0.9 +/- 0.8 L, maximum inspiratory pressure 25 +/- 16 cmH2O, maximum expiratory pressure 26 +/- 22 cmH2O and unassisted peak cough flow 169 +/- 90 L x min(-1). The greatest increase in peak cough flow was observed with mechanical insufflation/exsufflation at 235 +/- 111 L x min(-1) (p<0.01). All techniques showed similar patient acceptability. Mechanical insufflation/exsufflation produces a greater increase in peak cough flow than other standard cough augmentation techniques in adults and children with neuromuscular disease.
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              Peak flow and peak cough flow in the evaluation of expiratory muscle weakness and bulbar impairment in patients with neuromuscular disease.

              To study the expiratory muscle force and the ability to cough estimated by the peak expiratory flow and peak cough flow in patients with Duchenne muscular dystrophy and amyotrophic lateral sclerosis. A total of 27 patients with amyotrophic lateral sclerosis and 52 patients with Duchenne muscular dystrophy were studied. From the group of 144 normal subjects of this laboratory, we selected 38 for comparison. The maximal inspiratory pressure in patients with Duchenne muscular dystrophy and amyotrophic lateral sclerosis was 64.5 +/- 24.7% and 37.8 +/- 21.8%, respectively, and maximal expiratory pressure was 64.2 +/- 32.5% and 37.7 +/- 21.6%, respectively. Patient groups showed a significant lower peak expiratory flow than normal subjects. Higher peak cough flow than peak expiratory flow was found in all groups. The peak cough flow-peak expiratory flow difference was 46 +/- 18% in normal subjects, 43 +/- 23% in patients with Duchenne muscular dystrophy, and 11 +/- 17% in patients with amyotrophic lateral sclerosis. The peak expiratory flow and peak cough flow were not different in bulbar onset amyotrophic lateral sclerosis. In patient groups, the dynamic and static behavior correlated positively. These results suggest that peak cough flow-peak expiratory flow is useful to monitor expiratory muscle weakness and bulbar involvement and to assess its evolution in these patients.
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                Author and article information

                Journal
                Yonsei Med J
                YMJ
                Yonsei Medical Journal
                Yonsei University College of Medicine
                0513-5796
                1976-2437
                30 April 2005
                30 April 2005
                : 46
                : 2
                : 233-238
                Affiliations
                Department of Physical Medicine & Rehabilitation, Rehabilitation Institute of Muscular Disease, Yongdong Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.
                Author notes
                Reprint address: requests to Dr. Seong Woong Kang, Department of Physical Medicine & Rehabilitation, Yongdong Severance Hospital, 146-92 Dogok-dong, Kangnam-gu, Seoul 135-720, Korea. Tel: 82-2-3497-3492, Fax: 82-23463-7585, kswoong@ 123456yumc.yonsei.ac.kr
                Article
                10.3349/ymj.2005.46.2.233
                2823019
                15861496
                94299f17-6dd9-4a5f-9bc2-8946e7762248
                Copyright © 2005 The Yonsei University College of Medicine

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                Categories
                Original Article

                Medicine
                maximum insufflation capacity,pulmonary compliance,peak cough flow,assisted cough
                Medicine
                maximum insufflation capacity, pulmonary compliance, peak cough flow, assisted cough

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