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ABSTRACT: Background
Prevention and management of end-organ disease represent major challenges facing providers of children and adults with sickle cell disease (SCD). Uncertainty and variability in the screening, diagnosis, and management of cardiopulmonary and renal complications in SCD lead to varying outcomes for affected individuals.Objective
These evidence-based guidelines of the American Society of Hematology (ASH) are intended to support patients, clinicians, and other health care professionals in their decisions about screening, diagnosis, and management of cardiopulmonary and renal complications of SCD.Methods
ASH formed a multidisciplinary guideline panel that included 2 patient representatives and was balanced to minimize potential bias from conflicts of interest. The Mayo Evidence-Based Practice Research Program supported the guideline development process, including performing systematic evidence reviews up to September 2017. The panel prioritized clinical questions and outcomes according to their importance for clinicians and patients. The panel used the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach, including GRADE evidence-to-decision frameworks, to assess evidence and make recommendations, which were subject to public comment.Results
The panel agreed on 10 recommendations for screening, diagnosis, and management of cardiopulmonary and renal complications of SCD. Recommendations related to anticoagulation duration for adults with SCD and venous thromboembolism were also developed.Conclusions
Most recommendations were conditional due to a paucity of direct, high-quality evidence for outcomes of interest. Future research was identified, including the need for prospective studies to better understand the natural history of cardiopulmonary and renal disease, their relationship to patient-important outcomes, and optimal management.
SUBMITTER: Liem RI
PROVIDER: S-EPMC6963257 | biostudies-literature | 2019 Dec
REPOSITORIES: biostudies-literature
Liem Robert I RI Lanzkron Sophie S D Coates Thomas T DeCastro Laura L Desai Ankit A AA Ataga Kenneth I KI Cohen Robyn T RT Haynes Johnson J Osunkwo Ifeyinwa I Lebensburger Jeffrey D JD Lash James P JP Wun Theodore T Verhovsek Madeleine M Ontala Elodie E Blaylark Rae R Alahdab Fares F Katabi Abdulrahman A Mustafa Reem A RA
Blood advances 20191201 23
<h4>Background</h4>Prevention and management of end-organ disease represent major challenges facing providers of children and adults with sickle cell disease (SCD). Uncertainty and variability in the screening, diagnosis, and management of cardiopulmonary and renal complications in SCD lead to varying outcomes for affected individuals.<h4>Objective</h4>These evidence-based guidelines of the American Society of Hematology (ASH) are intended to support patients, clinicians, and other health care p ...[more]