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Respiratory pattern in an adult population of dystrophic patients
- Publication Year :
- 2011
-
Abstract
- We studied respiratory function and Chest Wall kinematics in a large population of adult patients affected by slow course muscular dystrophies such as Limb-Girdle Muscular Dystrophy (LGMD, n=38), Becker Muscular Dystrophy (BMD, n=20) and Facio-Scapulo Humeral Dystrophy (FSHD, n=30), through standard spirometry and through the Optoelectronic Plethysmography, to measure the thoraco-abdominal motion during Quiet Breathing and Slow Vital Capacity maneuvers. Within the restrictive pulmonary syndrome characterizing LGMD and FSHD, several different thoraco-abdominal patterns compared to those of healthy subjects were present in the more advanced stages of the disease. These differences were present in the seated position, during the execution of a maximal maneuver such as Slow Vital Capacity. A global respiratory (both inspiratory and expiratory) muscle involvement was more pronounced in the LGMD and FSHD than in the BMD patients, and a significant reduction of abdominal contribution in wheelchair bound patients was observed. In conclusion, OEP technique is able to reveal mild initial modifications in the respiratory muscles in FSHD and LGMD patients, which could be helpful for functional and new therapeutic strategy evaluation.
- Subjects :
- Adult
Lung Diseases
Male
musculoskeletal diseases
Spirometry
medicine.medical_specialty
Heart Diseases
Vital Capacity
Scoliosis
Muscular Dystrophies
Pulmonary function testing
Young Adult
Internal medicine
Humans
Medicine
Respiratory function
Muscle Strength
Respiratory system
Muscular dystrophy
Muscle, Skeletal
medicine.diagnostic_test
business.industry
Respiration
Dystrophy
Middle Aged
medicine.disease
Respiratory Function Tests
Plethysmography
Neurology
Cardiology
Physical therapy
Female
Neurology (clinical)
business
Limb-girdle muscular dystrophy
Subjects
Details
- Language :
- English
- Database :
- OpenAIRE
- Accession number :
- edsair.doi.dedup.....834bcf3fd5faa54bb5755db4bc36f9d4