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Background Respiratory dysfunction in Late Onset Pompe Disease (LOPD) is attributed primarily to diaphragm weakness; it is not always proportional to skeletal muscle weakness. Beyond diaphragm and rib cage muscles, we know that posterior trunk muscles participate to inspiration, and abdominal wall muscles contribute to forced expiration. We aimed to investigate whether the involvement of axial muscles detected by muscle MRI may correlate with respiratory dysfunction or influence respiratory functional tests. Methods In 19 patients with LOPD in different stages of disease, we analyzed trunk muscle MRI and upright forced vital capacity FVC, postural drop in VC, and maximal inspiratory and expiratory pressures (MIP, MEP). Results While upright FVC did not correlate with trunk muscle involvement, postural drop in VC, reflecting diaphragm weakness, was strongly influenced by the severity of involvement of all posterior and anterior muscles. Conclusion Trunk muscles involvement in LOPD may reveal respiratory dysfunction and contribute to postural drop in VC. It is likely that axial muscle weakness may impair the compensatory mechanisms occurring in clinostatism, and mainly operated by the abdominal muscles. Detection of axial muscle damage by MRI may thus suggest the need of more extensive respiratory assessment, i.e. by polysomnography, even when upright VC is still within normal ranges.

BACKGROUND: Until recently, it has been considered essential to maintain the use of a double-limb circuit in patients with amyotrophic lateral sclerosis (ALS) to avoid rebreathing expired air during invasive mechanical ventilation. Currently, life-sustaining home ventilators can work with a single, lighter circuit that is easier to manage. Our aim was to evaluate the effectiveness and safety of a single-limb circuit with intentional leaks (passive circuit) in comparison with a circuit with an expiratory valve (active circuit), in subjects with ALS who use invasive home ventilation. METHODS: We conducted a retrospective single-center study. The enrolled subjects were divided into 2 groups according to the type of exhalation port. The aim of the study was to compare arterial blood gases; nocturnal oxygen saturation recordings; and the occurrence of adverse events, both clinical and technical events. In addition, we compared the rate of mortality and unplanned hospital admissions that occurred within a year after discharge from the hospital. RESULTS: Forty-three subjects were included in our study: 23 who used a passive circuit and 20 who used an active circuit. No significant difference in nocturnal and diurnal gas exchanges was detected. The incidence of adverse events was significantly higher in the active circuit group (85% in active circuit vs 30% in passive circuit, P CONCLUSIONS: The passive circuit was shown to be as effective and safe as the active circuit during home invasive ventilation in the subjects with amyotrophic lateral sclerosis. A future randomized controlled study is necessary to confirm these results and to extend indications to other pathologies.

Objective: Only a limited percentage of persons with MS (pwMS) participate to multidisciplinary rehabilitation (MDR) because of poor support, knowledge and motivation. We reasoned that pwMS should be more effectively prepared to increase their adherence. We implemented an innovative collaborative approach, called “brief highimpact preparatory experience” (b-HIPE), inspired by an overarching model based on the interplay between competence, motivation and opportunity to increase in a short time awareness and motivation of pwMS. Methods: B-HIPE integrates physiotherapy, mindfulness, sailing, healthy diet, and cultural activities to be experienced in a convivial form at a beautiful seaside location in Sardinia. Sixteen pwMS participated to 3 successive one-week editions of the b-HIPE, co-sponsored by the Rotary Club of Milan and supported by researchers of our Institute and of partner associations. The feasibility was assessed with structured questionnaires and free reports concerning accommodation, logistics, coordination, social climate and the specific activities proposed. For this pilot study we used a single-group design with repeated measurements at baseline and post-intervention. The SF-36 QoL scale was the main outcome measure, the Fatigue Severity Scale (FSS), the Berg Balance scale (BBS) and the 9 hole peg test (9HPT) were the secondary outcomes. Results: The approach was feasible. Scores on several FS-36 scales and secondary outcomes were significantly improved. Participants’ satisfaction with all aspects of the experience was above expectations. PwMS became more motivated and aware of physical and mental resources, all learned to sail adapted monohulls, strategies to master stress and to modify their diet according to specific recommendations. Conclusion: B-HIPE is safe and feasible. The interplay of multiple factors produced in a very short time the expected changes in participants’ attitude toward a healthier lifestyle. A monitoring program is ongoing to assess long-term effects including adherence to hospital-based MDR. Publisher PDF

Aim. To evaluate in a Pulmonary Rehabilitation (PR) setting the prevalence of airflow obstruction (AO) in either current or former smokers ≥45 years old both with dyspnoea and with chronic productive cough, using European Respiratory society (ERS) statement (FEV1/SVC

Grading the severity of obstruction in patients with Chronic Obstructive Pulmonary Disease and morbid obesity. N. Barbarito, E. De Mattia. Aim. To evaluate the severity of airway obstruction in patients affected by chronic obstructive pulmonary dis- ease (COPD) in the presence of concomitant restriction due to morbid obesity. Methods. Lung function test, six-minute walking distance (6MWD) test, body mass index measurement (BMI), and de- termination of dyspnoea using the Modified Medical Re- search Council Dyspnoea Scale (MMRC) were performed on each patient referred to our department according to their in- dividual respiratory diagnosis or symptoms. Analysis was performed on smokers or ex-smokers patients, with both dys- pnoea and chronic productive cough, showing non fully re- versible airflow obstruction, with normal-weight (NW: BMI 22 to 24 kg/m2) or morbid-obesity (MO: BMI * 40 kg/m2). Results. In 33 COPD patients, spirometric data dif- fer between NW and MO only in fixed FEV1/FVC ratio (50±9 and 62±7, respectively; p = 0.0001) and FEV1/SVC % of predicted (57±15 and 71±11, respectively; p = 0.005). Furthermore, SVC was found to exceed FVC on- ly in NW (2.82±0.7 L and 2.08±0.9 L, respectively; p = 0.03). NW and MO differ significantly also in MMRC (3.4±0.9 vs 2.4±1, respectively; p = 0.004), 6MWD in me- tres (226±100 and 331±110, respectively, p = 0.007), 6MWD as % predicted (49±22 and 81±23, respectively; p = 0.0003), and BODE index (5.8±2 and 3.6±2, respec- tively; p = 0.003). Conclusions. There is a significant overgrading of ob- struction in morbidly obese patients affected by COPD. Therefore, we suggest that an alternative grading system be used for patients with mixed ventilatory dysfunction. Monaldi Arch Chest Dis 2013; 79: 3-4, 121-127.

Intrapulmonary percussive ventilation (IPV) is a ventilatory technique that delivers bursts of high-flow respiratory gas into the lung at high rates, intended for treating acute respiratory failure and for mobilization of secretions. We performed a study, aimed at assessing the physiological response to IPV, on patients' breathing pattern, inspiratory effort, lung mechanics and tolerance to ventilation. Ten COPD patients underwent randomized trials of IPV through a face mask at different pressure/frequency combinations (1.2 bar/250 cycles/min; 1.8/250; 1.2/350; 1.8/350), separated by return to baseline (SB), using the IMP2 ventilator. In 5 patients we have also compared the physiological changes of IPV with those obtained during pressure support ventilation (PSV). Minute ventilation did not vary among the trials, but tidal volumes (VT) were significantly greater during 1.2/250, 1.2/350 and 1.8/350 compared to SB. The pressure time product of the diaphragm per minute (PTPdi/min) estimate of the diaphragm oxygen expenditure was also significantly reduced during 1.2/250 and 1.8/250 (209 cmH2O x s/min for SB vs. 143 and 125 for 1.2/250 and 1.8/250, respectively P < 0.05), as well as dynamic intrinsic end-expiratory pressure (PEEPi,dyn). Similar reduction in PTPdi/min were obtained also during PSV. Tolerance to ventilation and oxygen saturation were satisfactory and did not change during the different trials. In 5 normal subjects a prolonged apnea trial lasting > 2 min was also performed, without any significant decrease in SaO2 or subjective discomfort. In conclusion, IPV was able to guarantee an adequate ventilation, while inducing a significant unloading of the diaphragm during the "low-frequency" trials

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Mechanical ventilation (MV) is a tool used to support the respiratory system. Non-invasive mechanical ventilation (NIMV) consists of the provision of MV via nasal mask or oro-nasal (face) mask, i.e., without the need for an invasive airway such as an endotracheal tube or tracheostomy cannula. The use of NIMV reduces or minimizes the short- and long-term intubation-related complications, such as laryngeal or tracheal injury, development of false airways, the need for heavy sedation or curarization causing generalized myopathy, and infective complications that are responsible for longer hospital stay and an excess in mortality [1–4].