Objectives: Neuromuscular respiratory failure after cervical spinal cord injury (cSCI) can lead to dependence on an invasive mechanical ventilator. Ventilator-free breathing after cSCI is associated with improved morbidity, mortality, and quality of life. We investigated the use of diaphragm muscle ultrasound to predict ventilator weaning outcomes after cSCI.
View Article and Find Full Text PDFIntroduction/aims: Phrenic neuropathy (PhN) impairs diaphragm muscle function, causing a spectrum of breathing disability. PhN etiologies and their natural history are ill-defined. This knowledge gap hinders informed prognosis and management decisions.
View Article and Find Full Text PDFInsufficient sleep syndrome, shift work disorder, and obstructive sleep apnea (OSA) not only significantly impact the health of affected individuals, but also pose a threat to public safety. This article describes the clinical manifestations and impact of these sleep disorders, particularly as they pertain to workers' health and those with safety-sensitive positions. Sleep deprivation, circadian rhythm disruptions, and excessive daytime sleepiness-hallmarks of insufficient sleep, shift work disorder, and OSA, respectively-all lead to a series of cognitive deficits and impaired concentration that affect workers in a wide variety of fields.
View Article and Find Full Text PDFWe diagnosed 66 peripheral nerve injuries in 34 patients who survived severe coronavirus disease 2019 (COVID-19). We combine this new data with published case series re-analyzed here (117 nerve injuries; 58 patients) to provide a comprehensive accounting of lesion sites. The most common are ulnar (25.
View Article and Find Full Text PDFUnlabelled: The coronavirus disease 2019 (COVID-19) pandemic is a reminder that global infectious disease outbreaks are not new and they have the potential to cause catastrophic morbidity and mortality, disrupt health care delivery, demand critical decision making in the absence of scientific certainty, interrupt trainee education, inflict economic damage, and cause a spike in demand for health care services that exceeds societal capacity. In this article, we look back at how the sleep medicine community adapted to challenges imposed by the COVID-19 pandemic. To mitigate viral transmission perhaps the single most effective and efficient adaptation was the rapid adoption of telemedicine.
View Article and Find Full Text PDFThe existing coverage criteria for noninvasive ventilation (NIV) do not recognize the benefits of early initiation of NIV for those with thoracic restrictive disorders and do not address the unique needs for daytime support as the patients progress to ventilator dependence. This document summarizes the work of the thoracic restrictive disorder Technical Expert Panel working group. The most pressing current coverage barriers identified were: (1) delays in implementing NIV treatment; (2) lack of coverage for many nonprogressive neuromuscular diseases; and (3) lack of clear policy indications for home mechanical ventilation (HMV) support in thoracic restrictive disorders.
View Article and Find Full Text PDFMany survivors from severe coronavirus disease 2019 (COVID-19) suffer from persistent dyspnea and fatigue long after resolution of the active infection. In a cohort of 21 consecutive severe post-COVID-19 survivors admitted to an inpatient rehabilitation hospital, 16 (76%) of them had at least one sonographic abnormality of diaphragm muscle structure or function. This corresponded to a significant reduction in diaphragm muscle contractility as represented by thickening ratio (muscle thickness at maximal inspiration/end-expiration) for the post-COVID-19 compared to non-COVID-19 cohorts.
View Article and Find Full Text PDFThe diaphragm, the principle muscle of inspiration, is an under-recognized contributor to respiratory disease. Dysfunction of the diaphragm can occur secondary to lung disease, prolonged ventilation, phrenic nerve injury, neuromuscular disease, and central nervous system pathology. In light of the global pandemic of coronavirus disease 2019 (COVID-19), there has been growing interest in the utility of ultrasound for evaluation of respiratory symptoms including lung and diaphragm sonography.
View Article and Find Full Text PDFWeaning to noninvasive ventilation in intensive care unit and bridging the patients to home with respiratory support is evolving as the technology of noninvasive ventilation is improving. In patients with chronic obstructive pulmonary disease exacerbation, timing of initiation of noninvasive ventilation is the key, as persistently hypercapnic patients show benefits. High-intensity pressure support seems to do better in comparison to low-intensity pressure support.
View Article and Find Full Text PDFChildren with rare genetic diseases that cause respiratory dysregulation are at particularly high mortality risk due to development of respiratory failure. The tectonin β-propeller-containing protein 2 (TECPR2) mutations are proposed to cause autophagy defect affecting axonal integrity and development of progressive neurodegenerative and neuromuscular disease. Published TECPR2 mutation cases have described a high prevalence of respiratory failure.
View Article and Find Full Text PDFBackground: A high prevalence of sleep disordered breathing (SDB) has been reported in persons with Marfan syndrome (MFS), a single gene disorder of connective tissue resulting in premature death from aortic rupture. The burden of SDB and accompanying hemodynamic stress could warrant broad screening in this population. Our goal was to assess the utility of traditional SDB screening tools in our sample of persons with MFS.
View Article and Find Full Text PDFThe role of noninvasive positive pressure ventilation (NIV) in severe chronic obstructive pulmonary disease (COPD) has been controversial. Over the past two decades, data primarily obtained from Europe have begun to define the clinical characteristics of patients likely to respond, the role of high-intensity NIV, and the potential best timing of initiating therapy. These approaches, however, have not been validated in the context of the U.
View Article and Find Full Text PDFBackground And Objective: Patients with chronic respiratory failure are increasingly managed with domiciliary non-invasive ventilation (NIV). There may be limited ability to provide NIV titration for these complex patients, and ventilatory requirements and upper airway support needs may change over time. Therefore, an automatically adjusting expiratory positive airway pressure (AutoEPAP) algorithm may offer advantages over manually adjusted EPAP for treating these patients.
View Article and Find Full Text PDFRespir Med Case Rep
February 2019
Background: Mirabegron, a β3 adrenergic receptor agonist, is FDA approved for treatment of overactive bladder. Approved in 2012 in the US, there have been no reports of any effects of mirabegron on pulmonary function.
Case Presentation: We report the case of a 65 year old male with a history of Parkinson's disease, OSA, and aspiration pneumonia presenting with subacute worsening dyspnea and found to have worsening restrictive ventilatory defect with a pattern consistent with neuromuscular weakness.
In 2010, Care Considerations for Duchenne Muscular Dystrophy, sponsored by the Centers for Disease Control and Prevention, was published in , and in 2018, these guidelines were updated. Since the publication of the first set of guidelines, survival of individuals with Duchenne muscular dystrophy has increased. With contemporary medical management, survival often extends into the fourth decade of life and beyond.
View Article and Find Full Text PDFCircadian misalignment is hypothesized to contribute to increased diabetes and obesity among shift workers and individuals with late sleep timing. Accordingly, the goal of our study was to identify-among normal and overweight/obese adults-associations between circadian timing (dim light melatonin onset; DLMO) and circadian misalignment (the interval between DLMO and sleep onset) with metabolic disease risk. This was a secondary analysis of data from a larger study.
View Article and Find Full Text PDFRationale: Amyotrophic lateral sclerosis (ALS) is a progressive neuromuscular disease resulting in respiratory failure and death. Use of noninvasive ventilation (NIV) improves survival. However, use of volume-assured pressure support (VAPS) has not been extensively studied in ALS.
View Article and Find Full Text PDFRationale: People with neuromuscular disease frequently have difficulty clearing pulmonary secretions, which leads to pneumonia and respiratory failure. High-frequency chest wall oscillation (HFCWO) is one intervention used to facilitate secretion clearance.
Objectives: The objective of this study was to determine if HFCWO therapy leads to improved outcomes as measured by lower healthcare use for patients who have a chronic neuromuscular disease.
As seen in this CME online activity (available at http://journal.cme.chestnet.
View Article and Find Full Text PDFPurpose Of Review: The focus of this review is to compare and contrast two orphan disorders of late-onset hypoventilation. Specifically, rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) and congenital central hypoventilation syndrome (CCHS) are distinct in presentation, pathophysiology, and etiology.
Recent Findings: While limited new information is available, appreciation and understanding of rare disorders can be attained through case reports.
There has been an exponential increase in the use of home noninvasive ventilation (NIV). Despite growing use, there is a paucity of evidence-based guidelines and practice standards in the United States to assist clinicians in the initiation and ongoing management of home NIV. Consequently, home NIV practices are being influenced by complicated local reimbursement policies and coding.
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