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Introduction: Tele-monitoring (TM) has proved effective in the home management of adult ventilator-dependent neuromuscular disease (NMD) patients. We aimed to evaluate a 2-year longitudinal multicentre TM trial designed for young ventilated NMD patients in terms of feasibility, home management of exacerbations and caregivers' burden.Methods: The TM trial protocol included patients' weekly scheduled overnight home-recording of SpO2, heart rate and ventilation and their transmission to each TM centre the following morning. Overnight data were reviewed by non-physicians and calls to families made to assess clinical condition. If clinical conditions (assessed by a scoring system) or overnight parameters worsened, either unscheduled transmissions or calls were activated and managed by non-physicians or medical team according to severity. Hospitalisations were compared with those of TM patients prior to TM start and with those of age-disease-severity-matched controls. Scores from the Caregiver Burden Inventory (CBI) questionnaire pre- and post-TM were compared.Results: Forty-eight patients were enrolled, 30 males, median age 16.4 years (interquartile range (IQR) 8.9-22.1), median ventilation/day 10.5 h (IQR 8-16). Exacerbations in TM patients did not differ (59 versus 53; p = 0.15) from controls. Hospitalisations were significantly reduced in TM patients when compared with those prior to TM (11 versus 24, p = 0.04) and to controls (11 versus 21, p = 0.03). Median hospitalisation length was significantly lower in TM patients than controls (6 versus 7 days, p = 0.03). Caregivers satisfaction was excellent whereas no significant changes in CBI were seen (32.5 versus 35.5, p = 0.06).Discussion: TM was effective in improving the home management of respiratory exacerbations in young ventilated NMD patients and overall well tolerated. [ABSTRACT FROM AUTHOR]

Background: This study aimed to show the impairment of autonomic cardiac conduction causing bradycardia and/or electrocardiographic alterations in children affected by spinal muscular atrophy type 1 and 2 (SMA 1 and 2). Methods: We included 25 spinal muscular atrophy patients, admitted from November 2016 to May 2017. All patients underwent an electrocardiographic examination and we studied PR and QRS intervals, P-waves and QRS amplitudes, and heart rate in spinal muscular atrophy patients compared to a control group. Results: In all patients, we found longer PRi and QRSi (P < .05), lower P-wave and QRS complex amplitudes (P < .01), and a decreased heart rate (P < .01) with respect to controls. When we divided our patients into SMA1 and SMA2 subgroups, we found that statistical differences were maintained for P-wave and QRS complex amplitudes and heart rate, but not for PRi and QRSi with respect to controls. Conclusion: We suggest the hypothesis of SMN expression on cardiac tissue condition and/or autonomic cardiac conduction. [ABSTRACT FROM AUTHOR]