Canadian respiratory journal : journal of the Canadian Thoracic Society
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The gene responsible for cystic fibrosis (CF) was discovered 25 years ago. This breakthrough has enabled a sophisticated understanding of how various mutations lead to specific alterations in the structure and function of the CF transmembrane regulator (CFTR) protein. ⋯ High-throughput drug screening approaches have yielded compounds that can modify CFTR structure and function, thus targeting the basic defect in CF. The present article describes the CFTR mutational classes, reviews mutation-specific therapies currently in late-phase clinical development, and highlights research opportunities and challenges with personalized medicine in CF.
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Observational Study
Corticosteroid therapy in critical illness due to seasonal and pandemic influenza.
Survey data suggest that Canadian intensivists administer corticosteroids to critically ill patients primarily in response to airway obstruction, perceived risk for adrenal insufficiency and hemodynamic instability. ⋯ Observational data revealed that hemodynamic instability and airway obstruction were associated with corticosteroid therapy in the critical care setting, similar to a recent survey of stated practice. Efforts to determine the effects of corticosteroids in the ICU for these specific clinical situations are warranted.
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Case Reports
Pediatric sand aspiration managed using bronchoscopy and extracorporeal membrane oxygenation.
Sand aspiration is a rare but potentially fatal occurrence to consider in near-drownings, accidental burials or cave-ins. Optimal management is not well defined.