Randomised trial of preventive nasal ventilation in Duchenne muscular dystrophy
Identifieur interne : 00CC36 ( Main/Exploration ); précédent : 00CC35; suivant : 00CC37Randomised trial of preventive nasal ventilation in Duchenne muscular dystrophy
Auteurs : Jean-Claude Raphael [France] ; Sylvie Chevret [France] ; Claude Chastang [France] ; Françoise Bouvet [France] ; the French Multicentre Cooperative Group on Home Mechanical Ventilation Assistance in Duchenne de Boulogne Muscular Dystrophy [France]Source :
- The Lancet [ 0140-6736 ] ; 1994.
English descriptors
- Teeft :
- Allelic variations, Arterial oxygen tension, Blood gases, Breathing room, Centre, Centre hospitalier universitaire, Compound muscle action, Compound nerve action potentials, Control group, Control group test, Conventional treatment, Delay death, Duchenne, Dystrophie musculaire, Dystrophy, Ethics committee, Genet epidemiol, Higher proportion, Human receptor, Interfascicular epineurium, Intermittent hyperinsufflation, Langue francaise, Leprous ulnar neuritis, Limit progression, Lutte contre, Mechanical ventilation, Muscular dystrophy, Nasal, Nasal mask, Necessary ventilation, Neuromuscular diseases, Nippv, Nippv group, Nocturnal nippv, Preventive nippv, Proc natl acad, Proximal, Proximal site, Pulmonary function, Pulmonary hyperinsufflation, Receptor, Reference date, Respir, Respiratory failure, Respiratory handicap, Respiratory insufficiency, Respiratory muscle weakness, Respiratory muscles, Respiratory muscles causes, Restrictive syndrome, Spinal roots, Surgical exposure, Tracheobronchial, Tracheobronchial secretions, Treatment comparison, Ulnar nerves, Ventilation, Ventilation assistance, Vital capacity.
Abstract
Abstract: Summary Duchenne muscular dystrophy (DMD) is the most common muscular dystrophy in children. Paralysis of respiratory muscles causes a decrease in forced vital capacity (FVC) from age 12 years, and death occurs between 20 and 25 years old and is usually related to respiratory insufficiency. Uncontrolled studies suggest that early home use of nasal intermittent positive-pressure ventilation (NIPPV) in DMD patients free of respiratory failure could limit progression of the restrictive syndrome and therefore improve survival Because efficacy of preventive NIPPV has not been demonstrated in a controlled trial, we undertook a randomised multicentre study in which 70 patients with DMD were included. Patients were free of daytime respiratory failure and FVC was between 20 and 50% of predicted values. At least 6 h of nocturnal NIPPV (n=35) was compared with conventional treatment (n = 35). During a mean follow-up of 52 months, 10 patients died, 8 in the NIPPV group and 2 in the control group (p=0·05, log-rank test). No differences were observed between the two groups for occurrence of hypercapnia, decrease of FVC below 20% of initial values, or use of necessary mechanical ventilation. Preventive NIPPV did not improve respiratory handicap and reduced survival of DMD patients. Use of NIPPV for preventive purposes should be avoided in patients with FVC between 20 and 50% of predicted values.
Url:
DOI: 10.1016/S0140-6736(94)93058-9
Affiliations:
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hyperinsufflation</term><term>Langue francaise</term><term>Leprous ulnar neuritis</term><term>Limit progression</term><term>Lutte contre</term><term>Mechanical ventilation</term><term>Muscular dystrophy</term><term>Nasal</term><term>Nasal mask</term><term>Necessary ventilation</term><term>Neuromuscular diseases</term><term>Nippv</term><term>Nippv group</term><term>Nocturnal nippv</term><term>Preventive nippv</term><term>Proc natl acad</term><term>Proximal</term><term>Proximal site</term><term>Pulmonary function</term><term>Pulmonary hyperinsufflation</term><term>Receptor</term><term>Reference date</term><term>Respir</term><term>Respiratory failure</term><term>Respiratory handicap</term><term>Respiratory insufficiency</term><term>Respiratory muscle weakness</term><term>Respiratory muscles</term><term>Respiratory muscles causes</term><term>Restrictive syndrome</term><term>Spinal roots</term><term>Surgical exposure</term><term>Tracheobronchial</term><term>Tracheobronchial secretions</term><term>Treatment comparison</term><term>Ulnar nerves</term><term>Ventilation</term><term>Ventilation assistance</term><term>Vital capacity</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Abstract: Summary Duchenne muscular dystrophy (DMD) is the most common muscular dystrophy in children. Paralysis of respiratory muscles causes a decrease in forced vital capacity (FVC) from age 12 years, and death occurs between 20 and 25 years old and is usually related to respiratory insufficiency. Uncontrolled studies suggest that early home use of nasal intermittent positive-pressure ventilation (NIPPV) in DMD patients free of respiratory failure could limit progression of the restrictive syndrome and therefore improve survival Because efficacy of preventive NIPPV has not been demonstrated in a controlled trial, we undertook a randomised multicentre study in which 70 patients with DMD were included. Patients were free of daytime respiratory failure and FVC was between 20 and 50% of predicted values. At least 6 h of nocturnal NIPPV (n=35) was compared with conventional treatment (n = 35). During a mean follow-up of 52 months, 10 patients died, 8 in the NIPPV group and 2 in the control group (p=0·05, log-rank test). No differences were observed between the two groups for occurrence of hypercapnia, decrease of FVC below 20% of initial values, or use of necessary mechanical ventilation. Preventive NIPPV did not improve respiratory handicap and reduced survival of DMD patients. Use of NIPPV for preventive purposes should be avoided in patients with FVC between 20 and 50% of predicted values.</div></front></TEI><affiliations><list><country><li>France</li></country><region><li>Île-de-France</li></region><settlement><li>Garches</li><li>Paris</li></settlement></list><tree><country name="France"><region name="Île-de-France"><name sortKey="Raphael, Jean Claude" sort="Raphael, Jean Claude" uniqKey="Raphael J" first="Jean-Claude" last="Raphael">Jean-Claude Raphael</name></region><name sortKey="Bouvet, Francoise" sort="Bouvet, Francoise" uniqKey="Bouvet F" first="Françoise" last="Bouvet">Françoise Bouvet</name><name sortKey="Chastang, Claude" sort="Chastang, Claude" uniqKey="Chastang C" first="Claude" last="Chastang">Claude Chastang</name><name sortKey="Chevret, Sylvie" sort="Chevret, Sylvie" uniqKey="Chevret S" first="Sylvie" last="Chevret">Sylvie Chevret</name><name sortKey="The French Multicentre Cooperative Group On Home Mechanical Ventilation Assistance In Duchenne De Boulogne Muscular Dystrophy" sort="The French Multicentre Cooperative Group On Home Mechanical Ventilation Assistance In Duchenne De Boulogne Muscular Dystrophy" uniqKey="The French Multicentre Cooperative Group On Home Mechanical Ventilation Assistance In Duchenne De Boulogne Muscular Dystrophy" first="" last="The French Multicentre Cooperative Group On Home Mechanical Ventilation Assistance In Duchenne De Boulogne Muscular Dystrophy">the French Multicentre Cooperative Group on Home Mechanical Ventilation Assistance in Duchenne de Boulogne Muscular Dystrophy</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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