Identifying Clinical and Research Priorities in Sickle Cell Lung Disease. An Official American Thoracic Society Workshop Report
| Autor: | Elizabeth K. Fiorino, Danna Tauber, Kim Smith-Whitley, Jennifer Knight-Madden, Joshua J. Field, Elizabeth S. Klings, Anastassios C. Koumbourlis, Julian L. Allen, Nancy Terry, Nargues Weir, Carol L. Rosen, Victor R. Gordeuk, Anne Greenough, Robyn T. Cohen, Alem Mehari, Dapa A Diallo, Thomas D. Coates, Benjamin T. Kopp, Folasade Ogunlesi, Claudia R. Morris, Mark T. Gladwin, Debra P Bennett, S. Christy Sadreameli, Sophie Lanzkron, Roberto F. Machado, A. Parker Ruhl, Jo Howard, Andrew D. Campbell, Robert I. Liem, LeRoy Graham, Swee Lay Thein, Gregory J. Kato, Jeffrey Glassberg, Elliott Vichinsky |
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| Rok vydání: | 2019 |
| Předmět: |
Pulmonary and Respiratory Medicine
medicine.medical_specialty Disease law.invention Pulmonary function testing 03 medical and health sciences 0302 clinical medicine Randomized controlled trial law pulmonary hypertension medicine 030212 general & internal medicine Intensive care medicine Pulmonologists American Thoracic Society Documents business.industry acute chest syndrome asthma medicine.disease Pulmonary hypertension Acute chest syndrome Natural history 030228 respiratory system sickle cell disease sleep disorders Observational study business |
| Zdroj: | Annals of the American Thoracic Society |
| ISSN: | 2325-6621 2329-6933 |
| DOI: | 10.1513/annalsats.201906-433st |
| Popis: | Background: Pulmonary complications of sickle cell disease (SCD) are diverse and encompass acute and chronic disease. The understanding of the natural history of pulmonary complications of SCD is limited, no specific therapies exist, and these complications are a primary cause of morbidity and mortality. Methods: We gathered a multidisciplinary group of pediatric and adult hematologists, pulmonologists, and emergency medicine physicians with expertise in SCD-related lung disease along with an SCD patient advocate for an American Thoracic Society–sponsored workshop to review the literature and identify key unanswered clinical and research questions. Participants were divided into four subcommittees on the basis of expertise: 1) acute chest syndrome, 2) lower airways disease and pulmonary function, 3) sleep-disordered breathing and hypoxia, and 4) pulmonary vascular complications of SCD. Before the workshop, a comprehensive literature review of each subtopic was conducted. Clinically important questions were developed after literature review and were finalized by group discussion and consensus. Results: Current knowledge is based on small, predominantly observational studies, few multicenter longitudinal studies, and even fewer high-quality interventional trials specifically targeting the pulmonary complications of SCD. Each subcommittee identified the three or four most important unanswered questions in their topic area for researchers to direct the next steps of clinical investigation. Conclusions: Important and clinically relevant questions regarding sickle cell lung disease remain unanswered. High-quality, multicenter, longitudinal studies and randomized clinical trials designed and implemented by teams of multidisciplinary clinician-investigators are needed to improve the care of individuals with SCD. |
| Databáze: | OpenAIRE |
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