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A Systematic Review of the Treatment of Chronic Rhinosinusitis in Adults with Primary Ciliary Dyskinesia

机译：系统性评价成人原发性睫状运动障碍的慢性鼻-鼻窦炎的治疗。

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Background: Primary ciliary dyskinesia (PCD) may be an underlying factor in some cases of refractory chronic rhinosinusitis (CRS). However, clinical management of this condition is not well defined. This systematic review examines the available evidence for the diagnosis and management of CRS in adults with PCD. Methods: A systematic review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. Pubmed, EMBASE, and Cochrane database were queried for studies pertinent to treatment of PCD in adults. Two investigators performed eligibility assessment for inclusion or exclusion in a standardized manner. Results: Of the 278 articles identified, six studies met the criteria for analysis. These studies had a predominately low level of evidence. Medical therapy included oral antibiotics and nasal saline rinses. Endoscopic sinus surgery (ESS) was described in three of six studies. Outcomes measures were limited and included non-validated questionnaires, subjective reporting of CRS symptoms, and decreased preciptins against pseudomonas following ESS. Recommendation for a standardized therapeutic strategy was not possible with the available literature. Conclusion: A paucity of evidence is available to guide the treatment of PCD in the adult population. Further prospective studies are needed to determine the optimal diagnostic and management strategy for this condition.
机译：背景：原发性睫状运动障碍（PCD）可能是某些难治性慢性鼻-鼻窦炎（CRS）的潜在因素。但是，这种情况的临床管理尚不明确。这项系统的审查检查了成人PCD的CRS诊断和治疗的可用证据。方法：根据系统评价和荟萃分析的首选报告项目（PRISMA）指南进行了系统评价。查询Pubmed，EMBASE和Cochrane数据库中与成人PCD治疗有关的研究。两名调查员以标准化方式对纳入或排除进行资格评估。结果：在确定的278篇文章中，有6项研究符合分析标准。这些研究的证据水平极低。医学治疗包括口服抗生素和鼻腔生理盐水冲洗。六项研究中的三项描述了内窥镜鼻窦手术（ESS）。结果的措施是有限的，包括未经验证的调查表，CRS症状的主观报告和ESS后假单胞菌的沉淀减少。现有文献无法推荐标准化治疗策略。结论：缺乏证据可指导成人人群PCD的治疗。需要进一步的前瞻性研究，以确定针对这种情况的最佳诊断和管理策略。

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