https://c.aarc.org/community/neuromuscular_roundtable/journal_club/112.1024.pdf

Prevention of Pulmonary Morbidity for Patients With Duchenne Muscular Dystrophy

https://journal.chestnet.org/article/S0012-3692(15)51214-2/pdf

Prevention of pulmonary morbidity for patients with neuromuscular disease.

The obesity hypoventilation syndrome can be treated with noninvasive mechanical ventilation.

Patients with neuromuscular disease (NMD) who require long-term ventilation normally have the ventilation set using empirical daytime parameters. We evaluated arterial blood gases (ABG), breathing pattern, respiratory muscle function, and sleep architecture during ventilation with two noninvasive Pressure Support Ventilation (nPSV) settings in nine patients with NMD. The two settings were randomly applied: the usual (US), with the nPSV setting titrated on simple clinical parameters, and the physiological (PHYS), tailored to the patient's respiratory effort. During wakefulness, nPSV significantly improved ABG and minute ventilation and reduced the diaphragmatic pressure-time product (PTPdi/breath), independently of the type of setting (PTPdi/breath spontaneous breathing 5.7 +/- 2.4, US 3.2 +/- 2, PHYS 3.6 +/- 1.6 cm H2O . seconds(-1), p < 0.001). However, during sleep, PHY nPSV resulted in a significant improvement of gas exchange, sleep efficiency (71.7% +/- 14 US vs. 80.6% +/- 8.3 PHYS, p < 0.01) and % of REM sleep (9.1% +/- 7 US vs. 17.3% +/- 5.4 PHYS, p < 0.01). This improvement was significantly correlated with the reduction in ineffective efforts. In NMD, nPSV is effective in improving daytime ABG and in unloading inspiratory muscles independently of whether it is set on the basis of the patient's comfort or the patient's respiratory mechanics. However, PHYS was associated with better sleep architecture and nighttime gas exchange.

Effects of Different Ventilator Settings on Sleep and Inspiratory Effort in Patients with Neuromuscular Disease

Neuromuscular diseases (NMD) may affect respiratory muscles, leading to respiratory failure. Studies show that long-term noninvasive mechanical ventilation (NIV) improves symptoms, gas exchange, quality of life and survival. NIV improved these parameters in muscular dystrophies and also in patients with amyotrophic lateral sclerosis without severe bulbar dysfunction. NIV should be started at the onset of nocturnal hypoventilation. In selected cases, NIV may be simpler, better accepted by patients and cheaper than invasive mechanical ventilation, but it cannot be used as an alternative. Tracheostomy may be preferred by patients unable to protect their airways and wishing to survive as long as possible, or by ventilator-dependent patients. Glossopharyngeal breathing consists of taking air and propelling it into the lungs. Chest percussions and vibrations can help to mobilise airway secretions but they cannot substitute coughing. Manually assisted coughing requires substantial lung inflation through air stacking or deep lung insufflation, followed by an abdominal thrust with open glottis. The combination of mechanical in-exsufflation with an abdominal thrust is a mechanically assisted cough. In conclusion, recent advances in respiratory care of NMD have improved prognosis and many caregivers have changed from a traditional noninterventional to a more aggressive, supportive approach.

https://erj.ersjournals.com/content/erj/34/2/444.full.pdf

Chronic respiratory care for neuromuscular diseases in adults

Nasal Intermittent Positive Pressure Ventilation: Analysis of Its Withdrawal

Fetal Lung Maturation IV: The Release of Phosphatidic Acid Phosphohydrolase and Phospholipids into the Human Amniotic Fluid

Juan M. Jimenez

Papers

Nasal Intermittent Positive Pressure Ventilation: Analysis of Its Withdrawal

Fetal lung maturation IV: the release of phosphatidic acid phosphohydrolase and phospholipids into the human amniotic fluid.

Phospholipid Biosynthesis : The Activity of Phosphatidic Acid Phosphohydrolase in the Developing Lung and Amnionic Fluid