![]() Patients who did not complete pulmonary function examination and refused to participate were excluded. Written informed consent was obtained from each participant. All the patients fulfilled the diagnostic criteria of ALS according to the revised Escorial criteria, and the diagnosis was confirmed by two professional neurologists. ![]() For the validation cohort, 62 newly diagnosed ALS patients were enrolled from Oct. ![]() In the present study, we aimed to screen the survival-related pulmonary function parameters, and subsequently explored the predictive value of PEF in disease severity and prognosis for ALS.įor the discovery cohort, 221 ALS patients were recruited serially from the Department of Neurology, First Affiliated Hospital of Fujian Medical University from Dec. Advantageously, PEF can be measured by a household peak flow meter, which is simple in structure, easy to use, cheap, and can be self-monitored at home. Peak expiratory flow (PEF) is another useful pulmonary function index, which was defined as the maximum expiratory flow per minute. So it is necessary to explore more practical and feasible pulmonary function indexes for the prediction of progression and survival in ALS. For those patients at the later stage of ALS, the pulmonary function tests are hardly carried out due to their unwillingness or inability to come to the hospital owing to their mobility inconvenience. For the time being, the pulmonary function tests are mainly performed by respiratory physiotherapists in hospitals with specialized instrumentation (MS-PFT spirometer), and largely depend on the cooperation of ALS patient. Forced vital capacity (FVC) is the most important respiratory index highly correlated with survival in ALS. Pulmonary function tests (PFTs) have been frequently applied for evaluating respiratory function. The assessment of respiratory function and respiratory care are therefore of great importance for ALS patients. Due to the rapidly progressive loss of motor function, most ALS patients die within 2 to 5 years after symptom onset, usually as a consequence of respiratory failure. Clinically, it is featured by adult onset, male predominance, upper limb muscle weakness and atrophy, and relentless progression involving trunk and bulbar muscles. PEF is an easily available pulmonary function index, which is also a promising indicator in predicting disease severity and survival for ALS patients.Īmyotrophic lateral sclerosis (ALS) is a chronic, incurable and fatal neurodegenerative disease, pathologically characterized by progressive loss of motor neurons in the motor cortex, brainstem, and spinal cord. Our work emphasizes the critical role of PFTs in predicting prognosis of ALS patients. Finally, we also confirmed that the values of KOKA-measured PEF were highly correlated with the ones measured using MS-PFT spirometer ( r = 0.9644, p < 0.0001). Besides, the values of PEF were positively associated with disease severity (ALSFRS-R score, r s = 0.539, P < 0.0001), and negatively associated with progression rate (ΔALSFRS-R, r s = -0.316, P < 0.0001). ResultsĪmong 12 pulmonary function parameters, FVC, FEV1, PEF, MEF75%, and MVV were identified to be independent predictive factors for survival. In the validation cohort, 62 newly diagnosed ALS patients performed the pulmonary function test by MS-PFT spirometer and household peak flow meter (KOKA) simultaneously. The discovery cohort included 202 ALS patients, and the demographic and clinical characteristics of eligible patients were collected and pulmonary function tests were performed using MS-PFT spirometer. In the present study, we aim to screen the survival-related pulmonary function parameters, and to explore the predictive value of peak expiratory flow (PEF) in disease severity and prognosis in patients with ALS. Amyotrophic lateral sclerosis (ALS) is an incurable and fatal neurodegenerative disease most ALS patients die within 3 to 5 years after symptom onset, usually as a consequence of respiratory failure.
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