Published ahead of print on March 30, 2006, doi:10.1164/rccm.200512-1841OC Am. J. Respir. Crit. Care Med., Volume 174, Number 1, July 2006, 67-74 A more recent version of this article appeared on July 1, 2006
Submitted on December 2, 2005 Respiratory Muscle Testing: A Valuable Tool for Children with Neuromuscular DisordersFrederic Nicot1,1 AP-HP, Hopital Armand Trousseau, Research Unit INSERM U 719, Universite Pierre et Marie Curie-Paris 6, Paris, France, 2 Lane Fox Respiratory Unit, Guy's and St. Thomas' NHS Foundation Trust, St. Thomas' Hospital, London, United Kingdom, 3 AP-HP, Hopital Armand Trousseau, Pediatric Physical Rehabilitation, Paris, France, 4 AP-HP, Pediatric Pulmonology Department, Hopital Armand Trousseau, Paris, France, 5 AP-HP, Hopital Armand Trousseau, Research Unit INSERM U 719, Universite Pierre et Marie Curie-Paris 6, Paris, France; AP-HP, Pediatric Pulmonology Department, Hopital Armand Trousseau, Paris, France, 6 Respiratory Muscle Laboratory, Royal Brompton Hospital, London, United Kingdom, 7 AP-HP, Department of Clinical Physiology, Hopital Raymond Poincare, Garches, France * To whom correspondence should be addressed. E-mail: brigitte.fauroux{at}trs.aphp.fr.
Rationale: Data on respiratory muscle performance in children with neuromuscular disorders is limited. Objectives: The aim of this study was to assess respiratory muscle strength by volitional and non-volitional tests and to compare these tests with forced vital capacity. Methods: Inspiratory muscle strength was assessed by measuring transdiaphragmatic and esophageal pressures generated during volitional and non-volitional maneuvers, whereas expiratory muscle strength was assessed by measuring the gastric pressure generated during a cough maneuver. Lung volumes were assessed by measuring forced vital capacity. Measurements and main results: Forty one patients with Duchenne muscular dystrophy (n=20), spinal amyotrophy (n=8), and congenital myopathy (n=13), were included, aged 2 to 18 years. All the patients were able to perform the sniff and the cough maneuver. Sniff transdiaphragmatic pressure decreased with age in Duchenne patients whereas it increased with age in patients with spinal amyotrophy and congenital myopathy. Magnetic stimulation of the phrenic nerves was obtained in all patients. Twenty five (61%) patients were able to perform forced vital capacity. In the three groups of patients, a positive correlation was observed between volitional, assessed by the sniff maneuver, and non-volitional respiratory muscle tests, assessed by the magnetic stimulation of the phrenic nerves. Also, forced vital capacity correlated with sniff transdiaphragmatic pressure and cough gastric pressure. Conclusions: Volitional respiratory muscle tests correlated with non-volitional tests and with forced vital capacity. Simple volitional respiratory muscle tests constitute a valuable tool for the assessment of respiratory muscle strength in young patients with neuromuscular disorders. Key words: Sniff maneuver, cough maneuver, Duchenne muscular dystrophy, spinal amyotrophy, congenital myopathy.
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