Annals of the American Thoracic Society
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Rationale: Lymphangioleiomyomatosis (LAM) is a female-predominant lung disease caused by mutations in the tuberous sclerosis complex (TSC) genes TSC1 and TSC2. Objectives: To examine the association between TSC mutation subtypes and the prevalence of LAM in women with TSC. Methods: Adult women seen at the Cincinnati Children's Hospital Medical Center's TSC clinic were stratified into the following three groups: those with TSC1 mutation, those with TSC2 mutation, and those with no mutation identified (NMI). ⋯ Serum VEGF-D (vascular endothelial growth factor-D) concentrations (median [95% confidence interval]) tended to be higher in patients harboring TSC2 mutations compared with patients with TSC1 mutations (725 pg/ml [612-1,317] vs. 331 pg/ml [284-406]; P = 0.03) and in patients with LAM compared with patients without LAM (725 pg/ml [563-1,609] vs. 429 pg/ml [357-773]; P = 0.02). Conclusions: LAM and angiomyolipomas are more common in women with TSC harboring TSC2 mutations compared with women with TSC1 mutations. Serum VEGF-D is a useful biomarker to suggest the presence of LAM in women with TSC.
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Many patients with obstructive sleep apnea (OSA) experience excessive daytime sleepiness (EDS), which can negatively affect daily functioning, cognition, mood, and other aspects of well-being. Although EDS can be reduced with primary OSA treatment, such as continuous positive airway pressure (CPAP) therapy, a significant proportion of patients continue to experience EDS despite receiving optimized therapy for OSA. This article reviews the pathophysiology and clinical evaluation and management of EDS in patients with OSA. ⋯ Patients who experience residual EDS despite primary OSA therapy may benefit from wake-promoting pharmacotherapy. Agents that inhibit reuptake of dopamine or of dopamine and norepinephrine (modafinil/armodafinil and solriamfetol, respectively) have demonstrated efficacy in reducing EDS and improving quality of life in patients with OSA. Additional research is needed on the effects of wake-promoting treatments on cognition in these patients and to identify individual or disorder-specific responses.
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Randomized Controlled Trial
Frequency and Risk Factors for Reverse Triggering in Pediatric ARDS during Synchronized Intermittent Mandatory Ventilation.
Rationale: Reverse triggering (RT) occurs when respiratory effort begins after a mandatory breath is initiated by the ventilator. RT may exacerbate ventilator-induced lung injury and lead to breath stacking. Objectives: We sought to describe the frequency and risk factors for RT among patients with acute respiratory distress syndrome (ARDS) and identify risk factors for breath stacking. ⋯ Together, these parameters were highly predictive of breath stacking (area under the curve, 0.979). Conclusions: Patients with higher tidal volume who have a set ventilator rate close to their spontaneous respiratory rate are more likely to have RT, which results in breath stacking >25% of the time. Clinical trial registered with ClinicalTrials.gov (NCT03266016).
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Rationale: During the coronavirus disease (COVID-19) pandemic, many intensive care units (ICUs) have shifted communication with patients' families toward chiefly telehealth methods (phone and video) to reduce COVID-19 transmission. Family and clinician perspectives about phone and video communication in the ICU during the COVID-19 pandemic are not yet well understood. Increased knowledge about clinicians' and families' experiences with telehealth may help to improve the quality of remote interactions with families during periods of hospital visitor restrictions during COVID-19. ⋯ Communication strategies suggested by families and clinicians for remote interactions include identifying a family point person to receive updates, frequently checking family understanding, positioning the camera on video calls to help family see the patient and their clinical setting, and offering time for the family and patient to interact without clinicians participating. Conclusions: Telehealth communication between families and clinicians of ICU patients appears to be a somewhat effective alternative when in-person communication is not possible. Use of communication strategies specific to phone and video can improve clinician and family experiences with telehealth.
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Observational Study
Persistent Post-COVID-19 Interstitial Lung Disease. An Observational Study of Corticosteroid Treatment.
Rationale: The natural history of recovery from severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) remains unknown. Because fibrosis with persistent physiological deficit is a previously described feature of patients recovering from similar coronaviruses, treatment represents an early opportunity to modify the disease course, potentially preventing irreversible impairment. Objectives: Determine the incidence of and describe the progression of persistent inflammatory interstitial lung disease (ILD) following SARS-CoV-2 when treated with prednisolone. ⋯ Conclusions: Following SARS-CoV-2 pneumonitis, a cohort of patients are left with both radiological inflammatory lung disease and persistent physiological and functional deficit. Early treatment with corticosteroids was well tolerated and associated with rapid and significant improvement. These preliminary data should inform further study into the natural history and potential treatment for patients with persistent inflammatory ILD following SARS-CoV-2 infection.