Current Opinion in Pulmonary Medicine

PURPOSE OF REVIEW: Advances in cystic fibrosis (CF) therapies over the past decade pivotally changed the morbidity and mortality of CF with the advent of cystic fibrosis transmembrane conductance regulator (CFTR) modulators that rescue dysfunctional CFTR protein in individuals with eligible genotypes. However, a significant proportion of the CF population is in need of alternative treatment strategies to address CFTR variants that are ineligible for therapeutic protein correction and/or potentiation...

PURPOSE OF REVIEW: Sleep disorders in Prader-Willi syndrome (PWS) range from respiratory to neurological disorders of sleep. We now recognize the role of excessive daytime sleepiness (present in the infant period and throughout life), and a modified narcolepsy phenotype with or without cataplexy. Disordered sleep in PWS may present with symptoms pervasive to daily function, including inattention at school, irritability, and behavioral outbursts. This review highlights the spectrum of sleep disordered breathing and neurological disorders of sleep in individuals with PWS as well as the current knowledge of management...

PURPOSE OF REVIEW: The nutritional landscape in cystic fibrosis has shifted dramatically in the era of CFTR modulator therapy. In this review, we will critically examine the literature on overweight and obesity in CF, current nutritional care unknowns and opportunities for further investigation or adaptation in clinical care. RECENT FINDINGS: Results of clinical trial and real-world data reflect marked improvement in nutritional status and quality of life. Clinical outcomes including CF related diabetes and CF related liver disease appear positively impacted...

PURPOSE OF REVIEW: Obstructive sleep apnea (OSA) is highly prevalent in patients with atrial fibrillation and plays a causal role for OSA in the pathogenesis of atrial fibrillation. The presence of OSA in atrial fibrillation is associated with increased symptom burden and increased risk of hospitalizations. Furthermore, untreated OSA is associated with an increased risk of atrial fibrillation recurrence post ablation or cardioversion, and observational studies suggest that continuous positive airway pressure (CPAP) therapy can attenuate this risk...

PURPOSE OF REVIEW: Highly effective modulators of cystic fibrosis transmembrane conductance regulator (CFTR) function have had dramatic impact on pulmonary and nutritional outcomes in persons with cystic fibrosis (pwCF). The impact on liver disease in pwCF was not the focus of the registration trials. The purpose of this review is to assess the current literature on the impact of HEMT on liver disease, progression, regression, and safety. RECENT FINDINGS: Short-term studies of HEMT in pwCF have shown that there is no significant impact on the frequency of liver enzyme abnormalities...

PURPOSE OF REVIEW: This review highlights the problem of neuropsychiatric adverse effects (AEs) associated with elexacaftor/tezacaftor/ivacaftor (ETI), current suboptimal mitigation approaches, a novel testable mechanistic hypothesis, and potential solutions requiring further research. RECENT FINDINGS: Studies show that a minority of persons with cystic fibrosis (PwCF) initiating cystic fibrosis transmembrane conductance regulator (CFTR) modulators experience neuropsychiatric AEs including worsening mood, cognition, anxiety, sleep, and suicidality...

PURPOSE OF REVIEW: Obstructive sleep apnoea (OSA) is an increasingly common pathology that all those involved in perioperative care will come across. Patients with the condition present a challenge at many stages along the perioperative journey, not least because many patients living with OSA are unaware of their diagnosis.Key interventions can be made pre, intra-, and postoperatively to improve outcomes. Knowledge of screening tools, diagnostic tests, and the raft of treatment options are important for anyone caring for these patients...

PURPOSE OF REVIEW: Pulmonary exacerbations are critical events with significant negative impacts in persons with cystic fibrosis, but their diagnosis and management are highly variable. Highly effective modulator therapies have greatly improved health and reduced exacerbation events, but have also reshaped how they present. This review discusses the complexities of the diagnosis and management of pulmonary exacerbations as well as the emerging work and evidence in this area. RECENT FINDINGS: The shifting epidemiology and our understanding of risk factors for pulmonary exacerbations are discussed...

PURPOSE OF REVIEW: Insomnia and obstructive sleep apnea have previously been viewed as completely independent conditions. However, there is now increasing recognition that insomnia and sleep apnea frequently co-occur. Co-morbid insomnia and sleep apnea (COMISA) is a highly prevalent condition that is associated with impairment of sleep, daytime function, mental health and physical health outcomes, and mortality risk. This review aims to provide an update on COMISA prevalence, consequences, treatment approaches, and future research directions...

PURPOSE OF REVIEW: To discuss the existing health inequities in people with cystic fibrosis (CF) and how the recent development of cystic fibrosis transmembrane regulator (CFTR) modulators may impact these inequities. RECENT FINDINGS: People with CF (pwCF) from low socioeconomic status (SES) have more pulmonary exacerbations, worse nutritional status, lower pulmonary function, and an increased mortality rate with less access to lung transplantation. pwCF who identify as racial and ethnic minorities have earlier mortality, lower lung function, are less likely to be detected on newborn screening resulting in a delayed diagnosis, are underrepresented in clinic trials, and less likely to be eligible for a CFTR modulator...

PURPOSE OF REVIEW: Cystic fibrosis is a genetic disease that increases risk of death from respiratory failure because of impairment in mucociliary clearance. Complex daily care regimens including medications and airway clearance techniques (ACTs) aim to preserve lung function and alleviate symptoms for people with cystic fibrosis (pwCF). The success of highly effective modulator therapy (HEMT) permits evaluation of treatment simplification. In this review, we evaluate adjustments made in daily respiratory care among pwCF taking HEMT and the feasibility of treatment simplification...

PURPOSE: Obstructive sleep apnea (OSA) severity varies considerably depending on the body position during sleep in certain subjects. Such variability may be underpinned by specific, body position-related changes in OSA pathophysiological determinants, or endotypes. Also head position relative to trunk may influence OSA endotypes. However, no studies to our knowledge have reviewed the endotype variations according to head or body position up to now. RECENT FINDINGS: Several findings illustrate that supine OSA is mostly attributable to unfavorable upper airway anatomy compared to lateral position...

PURPOSE OF REVIEW: Biannual clock changes to and from daylight saving time have been pervasive in many societies for over 50 years. Governments are considering abandoning this practice and choosing a single permanent time. RECENT FINDINGS: Our endogenous circadian clock follows our photoperiod, which changes over the year. The acute disruption caused by changing our clocks can affect safety (motor vehicle and on the job accidents), health (cardiovascular disease, drug overdoses, suicide), and human behavior (sport performance, generosity, and procrastination)...

PURPOSE OF REVIEW: Sarcoidosis is a multiorgan system disease exerting significant impact on biophysical, social, psychological and emotional well-being. Mortality and disability correlate to accessible, timely, expert care for sarcoidosis and its related complications. Across health conditions, positive healthcare interactions and interventions can rehabilitate unfavourable factors tied to concepts of 'frailty'. Here, we set out to introduce concepts related to frailty and their impact in the context of sarcoidosis...

PURPOSE OF REVIEW: Clinical presentation and outcomes in patients with sarcoidosis vary by race, gender, ethnicity, and geolocation. African Americans and female individuals have the highest incidence of disease. They are also more likely to present with advanced and more severe forms of disease, and to die from sarcoidosis. African American female individuals have the highest disease-associated mortality, yet this varies by geolocation. The diverse presentation and outcomes in sarcoidosis have often been attributed to genetics and biology, yet this may not be entirely so...

PURPOSE OF REVIEW: Pulmonary hypertension associated with interstitial lung disease (ILD-PH) is associated with significant alteration of quality of life, exercise capacity, and survival. Over the past 2 years, there were changes in the guideline definition and classification of ILD-PH, and positive randomized controlled trials were published. RECENT FINDINGS: Pulmonary hypertension associated with chronic lung disease is now hemodynamically defined as a mean pulmonary artery pressure more than 20 mmHg, with pulmonary artery wedge pressure 15 mmHg or less, and pulmonary vascular resistance (PVR) at least 2 Wood units...

PURPOSE OF REVIEW: This review provides an assessment of biomarkers in sarcoidosis, aiming to address the need for improved diagnostic, prognostic and management tools. Sarcoidosis presents diagnostic challenges, necessitating the search for reliable biomarkers to guide clinical decisions. RECENT FINDINGS: Established biomarkers such as serum angiotensin-converting enzyme (ACE) and serum interleukin-2 receptor (sIL-2R) have limitations in sensitivity and specificity...

PURPOSE OF REVIEW: Pulmonary Langerhans cell histiocytosis (PLCH) is a rare diffuse cystic lung disease that affects young to middle-aged smoking adults of both genders. The identification of molecular alterations in the canonical mitogen-activated protein kinase (MAPK) signalling pathway in most specific lesions has demonstrated the clonal/neoplastic nature of PLCH. We will summarize the progress made in the understanding of the pathogenesis of adult PLCH, and briefly highlight the recent findings useful for the management of the patients...

PURPOSE OF REVIEW: Genetics contributes substantially to the susceptibility to idiopathic pulmonary fibrosis (IPF). Genetic studies in sporadic and familial disease have identified several IPF-associated variants, mainly in telomere-related and surfactant protein genes.Here, we review the most recent literature on genetics of IPF and discuss how it may contribute to disease pathogenesis. RECENT FINDINGS: Recent studies implicate genes involved in telomere maintenance, host defence, cell growth, mammalian target of rapamycin signalling, cell-cell adhesion, regulation of TGF-β signalling and spindle assembly as biological processes involved in the pathogenesis of IPF...

PURPOSE OF REVIEW: The current review aims to highlight the role of primary care physicians in the diagnosis, treatment and monitoring of patients with sarcoidosis. Increased awareness of the clinical and imaging manifestations of the disease as well as the natural disease course will help for earlier and more accurate diagnosis as well as detection of high-risk patients who would benefit from treatment introduction. RECENT FINDINGS: Recent guidelines have attempted to deal with the confusion related to treatment indications, duration and monitoring of treatment in patients with sarcoidosis...