An unusual way to improve lung function in congenital myopathies: the power of singing.

Congenital myopathies (CMs) are a clinically and genetically heterogeneous group of disorders characterized by early onset weakness, hypotonia and characteristic structural abnormalities in muscle fibres. Hypotonia and weakness can be present at birth or appear in infancy, and a static or slowly progressive clinical course may present with muscle weakness, loss of spontaneous movement, involuntary muscle activity, and muscle atrophy. Often patients develop a restrictive syndrome and respiratory failure and require respiratory support In our case, we described lung improvement and respiratory muscle training due to singing in a young patient, affected by CMs with a poor adherence to non-invasive mechanical ventilation.

Remdesivir in Severe COVID-19 and Non-Invasive Ventilation: A Real-Life Experience.

BACKGROUND: Antiviral treatment is a hot topic regarding therapy for COVID-19. Several antiviral drugs have been tested in the months since the pandemic began. Yet only Remdesivir obtained approval after first trials. The best time to administer Remdesivir is still a matter for discussion and this could also depend upon the severity of lung damage and the staging of the infection. METHODS: We performed a real-life study of patients hospitalized forCOVID-19 and receiving non-invasive ventilation (NIV). In this single-center study, a 5 day course of Remdesivir was administered as compassionate use. Further therapeutic supports included antibiotics, low molecular weight heparin and steroids. Data collection included clinical signs and symptoms, gas exchange, laboratory markers of inflammation, and radiological findings. Major outcomes were de-escalation of oxygen-support requirements, clinical improvement defined by weaning from ventilation to oxygen therapy or discharge, and mortality. Adverse drug reactions were also recorded. All data were collected during hospitalization and during a 20-day follow up after treatment. RESULTS: 51 patients were enrolled. A global clinical improvement was recorded in 22 patients (43%) at 12 days, and 36 (71%) at 20 days; in particular, at 12 days, 27 patients (53%) also had a de-escalation of oxygen-support class from a therapeutic point of view. Remdesivir use was associated with a lower hazard ratio for clinical improvement in the elderly (older than 70 years) and in subjects with more extensive lung involvement (total severity score at HRCT of more than 14). The 20-day mortality was 13%. CONCLUSIONS: Results demonstrated that Remdesivir is associated with an improvement in clinical, laboratory and radiological parameters in patients with severe COVID-19 and showed an overall mortality of 13%. We conclude that, in this cohort, Remdesivir was a beneficial add-on therapy for severe COVID-19, especially in adults with moderate lung involvement at HRCT.

MAPK15-ULK1 signaling regulates mitophagy of airway epithelial cell in chronic obstructive pulmonary disease.

Airway epithelial mitochondrial oxidative stress and damage is an important pathology in chronic obstructive pulmonary disease (COPD). Mitophagy involves MAPK15-ULK1 signaling, the role of which is unknown in COPD. This study investigated MAPK15-ULK1 signaling in airway epithelial cells of COPD patients and its activation by cigarette smoke extract (CSE) in isolated human airway epithelial cells. Significant increased phosphorylation of MAPK15 and ULK1 (Ser555) was detected in the airway epithelium of COPD patients. This pathology was maintained in isolated primary COPD-epithelial cells. Compared to control cells, the protein expression of Beclin1 and the ratio of LC3II to LC3I were both significantly increased in COPD-epithelial cells. In human airway epithelial cells, CSE significantly increased the phosphorylation of MAPK15, ULK1 (Ser555), the expression of Beclin1, and the LC3II/LC3I ratio in a concentration- and time-dependent manner. Transfection with MAPK15 siRNA significantly inhibited the CSE-induced ULK1 (Ser555) phosphorylation in airway epithelial cells. Silencing of MAPK15 or ULK1 significantly reduced CSE-induced mitophagy and mitochondrial oxidative stress, thereby improving cell viability. In summary, cigarette smoke activated MAPK15-ULK1 signaling, thereby promoting mitophagy and mitochondrial oxidative stress in airway epithelial cells. This signaling pathway is activated in COPD-epithelial cells and therefore might present a novel therapeutic target for COPD.

Uncommon anomalies of the left pulmonary artery during VATS lobectomy for lung cancer.

The key to avoid intraoperative complications due to failure in the preoperative detection of pulmonary vascular anomalies is to perform a careful hilar dissection.

Multiple variations in pulmonary veins during a thoracoscopic right lower lobectomy: A case report.

A knowledge of pulmonary vein anatomy variants allows an appropriate preoperative radiological assessment and safe surgical management of vascular anomalies in patients undergoing major lung resections. In our case, multiple pulmonary vein variations were identified pre- and intraoperatively in a patient undergoing thoracoscopic right lower lobectomy and included superior and common basal veins from the right lower lobe draining separately into the left atrium, middle lobe veins joining the superior segment right lower lobe vein and additional superior segment right lower lobe vein draining directly into the left atrium. The recognition of these anatomical abnormalities in pulmonary veins may help thoracic surgeons avoid surgical complications in patients undergoing anatomical lung resections.

Effect of CArbocisteine in Prevention of exaceRbation of chronic obstructive pulmonary disease (CAPRI study): An observational study.

BACKGROUND: Chronic Obstructive Pulmonary Disease (COPD) is a chronic and progressive lung disease characterized by irreversible airflow obstruction, airway inflammation, oxidative stress and, often, mucus hypersecretion. The aim of this study is to determine if carbocisteine, a mucolytic and antioxidant agent, administered daily for 12 months, can reduce exacerbation frequency in COPD patients. METHODS: This observational study was conducted in Naples (population approximately 1000,000), Italy. It included 85 out-patients (mean age of 67.8 ± 8.6 years) followed by Clinic of Respiratory Diseases of the University Federico II. Every patient underwent spirometry demonstrating airflow obstruction not fully reversible according to ERS/ATS criteria for COPD diagnosis (Tiffenau index less than 70% after administration of salbutamol, a beta2 agonist drug). Patients enrolled had diagnosed COPD since 2 years and suffered at least one exacerbation in the previous year. None of the patients had been treated with carbocisteine or other mucolytic agent for a longer period of time than 7 days and no more than 4 times in the previous year to the enrollment. All of them assumed daily 2.7 g of carbocisteine lysine salt for a year in addition to their basic therapy. RESULTS: The comparison of exacerbation frequency between the previous year (T0) and the end of study treatment (T12), documents a statistically significant reduction of exacerbations(number of exacerbations at T0: 2 [1,3] vs number of exacerbations at T12: 1 [1,2]; p < 0.001).Quality of life was also reported and showed a statistically significant improvement at the end of the study (p < 0.001).We did not find correlation between reducing exacerbation frequency and exposure to cigarette smoking, passive smoking exposure in childhood, the use of inhaled steroids, the level of education of our patients and the GOLD stadium. INTERPRETATION: Daily administration of a mucolytic drug such as carbocisteine for prolonged periods in addition to the bronchodilator therapy can be considered a good strategy for reducing exacerbation frequency in COPD.