Catherine Thil1, Nelly Agrinier2, Bruno Chenuel1, Mathias Poussel1,3. 1. Department of Pulmonary Function Testing and Exercise Physiology, CHRU Nancy, Nancy, F-54000, France. 2. INSERM CIC-1433, CHRU Nancy, Nancy, F-54000, France. 3. EA 3450, Development, Adaptation and Disadvantage, Cardiorespiratory Regulations and Motor Control, Université de Lorraine, Nancy, France.
Abstract
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.
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 DM1patients 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.
Authors: Carola R Ferrari Aggradi; Elisa Falcier; Andrea Lizio; Alice Pirola; Jacopo Casiraghi; Alice Zanolini; Elena Carraro; Luca Mauro; Fabrizio Rao; Elisabetta Roma; Antonino Iannello; Elisa De Mattia; Andrea Barp; Sara Lupone; Valentina Gatti; Cristina Italiano; Valeria A Sansone Journal: Can Respir J Date: 2022-06-18 Impact factor: 2.130