RESUMEN
INTRODUCTION: Quality of life and prognosis among patients with myotonic dystrophy type 1 (DM1) depend on the degree of respiratory impairment. However, the changes over time in pulmonary function in DM1 have not been clearly described. METHODS: We retrospectively reviewed pulmonary function tests of 80 DM1 patients followed for at least 5 years. RESULTS: During 9.02 ± 3.4 years of follow-up, the average annual changes were: forced vital capacity (FVC) -0.034 ± 0.06 L (-0.72 ± 1.7% predicted); forced expiratory volume in 1 second (FEV1 ) -0.043 ± 0.05 L (-1.07 ± 1.7% predicted); and total lung capacity (TLC) -0.047 ± 0.1 L (-1.15 ± 1.7% predicted). CONCLUSIONS: These results suggest that, compared with other neuromuscular disorders, DM1 is, overall, associated with slowly progressive impairment of lung function. Muscle Nerve 56: 816-818, 2017.
Asunto(s)
Pulmón/fisiopatología , Distrofia Miotónica/diagnóstico , Distrofia Miotónica/fisiopatología , Adulto , Femenino , Estudios de Seguimiento , Humanos , Estudios Longitudinales , Masculino , Persona de Mediana Edad , Pruebas de Función Respiratoria/tendencias , Estudios RetrospectivosRESUMEN
We intended to assess the relationship between sleep strategies and performance during the North-Face Ultra-Trail du Mont-Blanc 2013, to test the hypothesis that sleep management can influence athletic performance. Almost all runners specifically adopted sleep management strategies before the race. Among the finishers 72% didn't sleep at all during the race and 28% took a least one break for sleep. Non-sleepers completed the race faster than the sleepers (P = 0.0008). Race time was positively correlated with drowsiness (P < 0.0001) and negatively correlated with the number participations in this race (P = 0.0039). Runners who adopted a sleep management strategy based on increased sleep time before the race completed the race faster (P = 0.0258). Most finishers seemed to be aware of the importance of developing sleep management strategies and increasing sleep time some nights before the race appeared to be the most relevant strategy to improve performance.
Asunto(s)
Rendimiento Atlético/fisiología , Resistencia Física/fisiología , Carrera/fisiología , Sueño/fisiología , Adulto , Conocimientos, Actitudes y Práctica en Salud , Humanos , Persona de Mediana Edad , Fases del Sueño/fisiología , Encuestas y Cuestionarios , Factores de TiempoRESUMEN
Myotonic dystrophy Type 1 (DM1) is the most common muscular dystrophy in adults. Respiratory failure is common but clinical findings support a dysregulation of the control of breathing at central level, furthermore contributing to alveolar hypoventilation independently of the severity of respiratory weakness. We therefore intended to study the relationship between the ventilatory response to CO2 and the impairment of lung function in DM1 patients. Sixty-nine DM1 patients were prospectively investigated (43.5 ± 12.7 years). Systematic pulmonary lung function evaluation including spirometry, plethysmography, measurements of respiratory muscle strength, arterial blood gas analysis and ventilatory response to CO2 were performed. Thirty-one DM1 patients (45%) presented a ventilatory restriction, 38 (55%) were hypoxaemic and 15 (22%) were hypercapnic. Total lung capacity decline was correlated to hypoxaemia (p = 0.0008) and hypercapnia (p = 0.0013), but not to a decrease in ventilatory response to CO2 (p = 0.194). Ventilatory response to CO2 was reduced to 0.85 ± 0.67 L/min/mmHg and not correlated to respiratory muscle weakness. Ventilatory response to CO2 was neither different among restricted/non-restricted patients (p = 0.2395) nor among normoxaemic/hypoxaemic subjects (p = 0.6380). The reduced ventilatory response to CO2 in DM1 patients appeared independent of lung function impairment and respiratory muscle weakness, suggesting a central cause of CO2 insensitivity.