American Society of Hematology 2019 guidelines for sickle cell disease: cardiopulmonary and kidney disease
American Society of Hematology 2019 guidelines for sickle cell disease: cardiopulmonary and kidney disease
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DOI：
10.1182/bloodadvances.2019000916
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发表时间：
2019-12-10
期刊：
影响因子：
7.5
通讯作者：
Mustafa, Reem A.
中科院分区：
文献类型：
--
作者：
Liem, Robert, I;Lanzkron, Sophie;Mustafa, Reem A.
背景：终末器官疾病的预防和管理是镰状细胞病（SCD）儿童和成人的提供者面临的主要挑战。 SCD 心肺和肾脏并发症的筛查、诊断和治疗的不确定性和可变性导致受影响个体的结局不同。 目的：美国血液学会 (ASH) 的这些循证指南旨在支持患者、临床医生和其他医疗保健专业人员做出关于 SCD 心肺和肾脏并发症的筛查、诊断和治疗的决策。方法：ASH 形成了一个多学科指南小组，其中包括 2 名患者代表，并进行了平衡，以最大程度地减少因不同观点之间的冲突而产生的潜在偏差。兴趣。梅奥循证实践研究计划支持指南的制定过程，包括截至 2017 年 9 月进行系统证据审查。专家组根据临床问题和结果对临床医生和患者的重要性对临床问题和结果进行优先排序。专家组使用建议分级评估、制定和评估 (GRADE) 方法（包括 GRADE 证据到决策框架）来评估证据并提出建议，并接受公众意见。 结果：专家组就 SCD 心肺和肾脏并发症的筛查、诊断和管理达成了 10 项建议。还制定了与患有 SCD 和静脉血栓栓塞的成人抗凝持续时间相关的建议。结论：由于缺乏有关感兴趣结果的直接、高质量证据，大多数建议都是有条件的。确定了未来的研究，包括需要进行前瞻性研究，以更好地了解心肺和肾脏疾病的自然史、它们与患者重要结果的关系以及最佳管理。
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.