American journal of respiratory and critical care medicine
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Am. J. Respir. Crit. Care Med. · Apr 2017
Lumacaftor/Ivacaftor in Patients Aged 6-11 Years With Cystic Fibrosis Homozygous for F508del-CFTR.
Combination lumacaftor/ivacaftor has been shown to improve lung function and other endpoints in patients aged 12 years and older with cystic fibrosis and homozygous for F508del-CFTR, but it has not been assessed in younger patients. ⋯ Lumacaftor/ivacaftor was well tolerated in this young population; no new safety concerns were identified. Improvements in lung clearance index, sweat chloride, nutritional status, and health-related quality of life were observed after 24 weeks of treatment. Clinical trial registered with www.clinicaltrials.gov (NCT01897233).
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Am. J. Respir. Crit. Care Med. · Apr 2017
Letter Randomized Controlled TrialProlonged Apnea Supported by High-Frequency Noninvasive Ventilation: A Pilot Study.
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Am. J. Respir. Crit. Care Med. · Apr 2017
Fifty Years of Research in ARDS. The Epidemiology of ARDS: A Fiftieth Birthday Review.
Since its first description 50 years ago, no other intensive care syndrome has been as extensively studied as acute respiratory distress syndrome (ARDS). Despite this extensive body of research, many basic epidemiologic questions remain unsolved. The lack of gold standard tests jeopardizes accurate diagnosis and translational research. ⋯ In this Critical Care Perspective, we discuss the historical context of ARDS description and attempts at its definition. We highlight the epidemiologic challenges of studying ARDS, as well as other intensive care syndromes, and propose solutions to address them. We update the current knowledge of ARDS trends in incidence and mortality, risk factors, and recently described endotypes.