[Neuromuscular disease: respiratory clinical assessment and follow-up].
Patients with neuromuscular disease are an important group at risk of frequently suffering acute or chronic respiratory failure, which is their main cause of death. They require follow-up by a pediatric respiratory medicine specialist from birth or diagnosis in order to confirm the diagnosis and treat any respiratory complications within a multidisciplinary context. The ventilatory support and the cough assistance have improved the quality of life and long-term survival for many of these patients. In this paper, the authors review the pathophysiology, respiratory function evaluation, sleep disorders, and the most frequent respiratory complications in neuromuscular diseases. The various treatments used, from a respiratory medicine point of view, will be analyzed in a next paper.
Sección de Neumología Pediátrica, Hospital Universitario La Paz, Madrid, España. Electronic address: cmcarrasco@salud.madrid.org.
C
C
Martínez Carrasco
Sección de Neumología Pediátrica, Hospital Universitario del Niño Jesús, Madrid, España.
J R
JR
Villa Asensi
Sección de Neumología Pediátrica, Hospital Materno Infantil Doce de Octubre, Madrid, España.
M C
MC
Luna Paredes
Sección de Neumología Pediátrica, Hospital Universitario Son Espases, Palma de Mallorca, España.
F B
FB
Osona Rodríguez de Torres
Sección de Neumología Pediátrica, Hospital Universitario Son Espases, Palma de Mallorca, España.
J A
JA
Peña Zarza
Sección de Neumología Pediátrica, Hospital Parc Taulí, Sabadell, Barcelona, España.
H
H
Larramona Carrera
Sección de Neumología Pediátrica, Hospital Sant Joan de Déu, Barcelona, España.
J
J
Costa Colomer
D002648:Child; D003680:Deglutition Disorders / Q000209:etiology; D005500:Follow-Up Studies; D006801:Humans; D009468:Neuromuscular Diseases / Q000145:classification / Q000150:complications*; D012120:Respiration Disorders / Q000175:diagnosis / Q000209:etiology*; D013147:Spirometry
Atrofia muscular espinal; Chronic respiratory failure; Distrofia muscular de Duchenne; Duchenne muscular dystrophy; Enfermedad neuromuscular; Insuficiencia respiratoria crónica; Neumología pediátrica; Neuromuscular disease; Non-invasive ventilation; Pediatric respiratory medicine; Sleep-disordered breathing; Spinal muscular atrophy; Trastorno respiratorio del sueño; Ventilación no invasiva