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61.
软骨发育不全是最常见的骨骼发育不良,以短肢、大头畸形、面中部发育不良和三叉戟手为特征。软骨发育不全相关的潜在并发症包括下肢长骨弯曲、中耳功能障碍、阻塞性睡眠呼吸暂停,以及更罕见的颈髓压迫、脑积水和胸腰椎后凸。软骨发育不全的管理是多方面的,需要在整个生命过程中多个学科共同参与。该文重点介绍了国内外近年来发表的软骨发育不全患者治疗和护理方面的指南或共识,旨在为临床医疗提供指导,以帮助识别软骨发育不全疾病有发生严重后遗症的高风险患者、并在并发症出现之前进行干预。  相似文献   
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为保障新型冠状病毒肺炎(COVID-19)突发公共卫生事件中药品供应稳定,2020年4月欧盟发布了“COVID-19持续期间的最优和合理药品供应以避免药品短缺的指导原则”。该指导原则提供了公共卫生事件期间,欧盟成员国维持药品供应稳定应遵循的工作建议;并针对COVID-19治疗用药品和因疫情影响而具有短缺风险的药品,提出维持药品供应、流通和使用的最优措施。介绍该指导原则的主要内容,以期为完善中国公共卫生体系建设提供参考。  相似文献   
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目的:采用循证评价和文献综述的方法,总结和分析目前已发表的儿童严重急性呼吸综合征冠状病毒2型(SARS-CoV-2)感染病例、临床实践指南或专家共识以及儿童严重急性呼吸综合征(SARS)/中东呼吸综合征(MERS)感染药物治疗经验,以期为儿童SARS-CoV-2感染药物治疗方案的制定提供循证依据。方法:系统检索PubMed、EMBase、the Cochrane Library、CNKI(含新型冠状病毒肺炎专题知识库)、WanFang Data、SinoMed和中华医学会杂志社(新型冠状病毒防控和诊治专栏)等数据库,根据文献研究特点和需要,设置快速检索策略和时限,对符合纳入标准研究中的药物治疗部分进行总结和描述性分析。结果:最终纳入11篇报告儿童病例的研究或综述(共196例患儿)和8篇指南,对儿童SARS/MERS感染的药物治疗经验进行综述。截至目前,已报道儿童感染病例的药物治疗方案基本依从儿童SARS-CoV-2感染指南推荐,且与既往发表的儿童SARS/MERS的药物治疗经验基本一致。α-干扰素为最常应用和最多推荐的抗病毒药物,其他抗病毒药物的应用多参考成人用法,有效性和安全性需要临床研究进一步予以证实;糖皮质激素和人免疫球蛋白在重症患儿中的应用尚存在争议,需权衡利弊谨慎使用;抗菌药物治疗需严格把握用药指征;其他治疗(如中医药)同样需要更多数据予以支持。结论:目前对于儿童SARS-CoV-2感染,尚无确证有效的药物治疗方案,这与儿童SARS/MERS的药物治疗经验基本一致,药物治疗方案的制定仍需进一步临床试验证据和用药经验总结的支持。  相似文献   
64.
Atopic dermatitis imposes a significant burden on patients, families and healthcare systems. Management is difficult, due to disease heterogeneity, co-morbidities, complexity in care pathways and differences between national or regional healthcare systems. Better understanding of the mechanisms has enabled a stratified approach to the management of atopic dermatitis, supporting the use of targeted treatments with biologicals. However, there are still many issues that require further clarification. These include the definition of response, strategies to enhance the responder rate, the duration of treatment and its regimen (in the clinic or home-based), its cost-effectiveness and long-term safety. The EAACI Guidelines on the use of dupilumab in atopic dermatitis follow the GRADE approach in formulating recommendations for each outcome and age group. In addition, future approaches and research priorities are discussed.  相似文献   
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The measurement of corrected count increment at 1-h post-transfusion (CCI-1 h) of platelet concentrate (PC) transfusion is recommended, but in the revised Japanese Guideline (2017) it was changed to “after 10-min to 1-h”, following the revision of the guidelines from Western countries. Here, we aimed to investigate on the feasibility to apply the CCI measured at 10-min or 30-min post-transfusion as the surrogate of CCI-1 h. Peripheral blood was collected at 10-min, 30-min and 1-h post-transfusion of PC and the effectiveness of the transfusion was analyzed based on the CCI. In the period from December 2017 to February 2020, 8 patients, who received multiple PC transfusion (total 208) at our institution, were analyzed. We performed the univariate analyses to examine the relationship between CCI value and the categorical variables, p-value <0.1 was obtained for gender (p = 2.91 × 10?19), fever after transfusion (p = 0.0163). The qualitative variables, namely measurement time (p = 0.0553), also showed p-value <0.1. Using these factors as covariates in the mixed effect model, we found that the measurement time (p = 0.0007) had a significant effect on the CCI value when looking at fixed effects. Although there is a tendency for decreased CCI values with time progression, the slope of the change in the mixed model was -0.00307, indicating that the CCI difference among the 3 measurements was small. Here we provide evidence that CCI measured at 10-min and 30-min post-transfusion give results comparable to those measured at 1-h post-transfusion, under the Japanese practice of platelet transfusion, which relies on 100 % single-donor apheresis PC, and ABO-identical whenever possible.  相似文献   
68.
目的:对某院使用华法林的住院患者进行用药分析和评价,并对其出院后复诊情况进行追踪,以更好地发挥华法林的治疗作用,减少不良反应的发生。方法:采用回顾性的分析方法,对某院2018年7月-12月使用华法林的住院患者用药进行分析,结合指南、共识对其用药情况进行分析评价。结果:共调取509份病例,华法林的使用主要分布于心血管内科、心血管外科、呼吸内科。华法林应用临床诊断主要为肺栓塞、心房颤动、肺动脉高压、静脉血栓等;住院患者华法林用量范围为0.625~9 mg,平均日剂量(2.99±1.11)mg;全院服用华法林后INR总体测定率为79%,将INR值以"<2.0"、"2.0~3.0"、">3.0"分布进行统计,INR2.0~3.0占比为33.17%,最高的为呼吸内科。26例(5.11%)患者住院期间INR达到4.5~10.0,38.46%使用维生素K进行拮抗;2例(0.39%)患者INR大于10,全部使用维生素K进行拮抗。在院期间合用其他抗凝药中,低分子肝素使用率最高的为骨外科,心血管外科,心血管内科;普通肝素使用率最高的为呼吸内科;阿加曲班使用率最高的为神经内科;利伐沙班使用率最高的为心血管内科,根据相关指南共识分析用药基本合理。患者出院后一周内及总复诊率最高的均为呼吸内科。复诊后INR分布在2.0~3.0占比最高的也为呼吸内科,与呼吸科临床药师药学监护及用药教育相关。结论:目前华法林临床应用仍不够规范,INR达标率偏低,出院后复诊率较低,抗凝药物转换过程中有不规范的问题,积极开展药师药学监护、用药教育及随访具有重要意义。  相似文献   
69.
2005年《NCCN直肠癌临床实践指南》专家组关于直肠癌的外科原则共有4项推荐,包括主刀医师术前亲自行内镜检查,切除原发肿瘤、保证足够切缘,切除肿瘤淋巴引流区以及尽可能保留器官结构的完整性.截至2015年,外科原则推荐意见共进行了4次更新,但是,始终要求术者应亲自进行内镜检查,以保证术者清晰掌握肿瘤范围、位置,并对手术方式选择、能否保留肛门做出合理的判断考量.回顾十年间《NCCN直肠癌临床实践指南》关于外科原则的推荐意见,专家组始终坚持以“追求R0切除和尽量保留器官结构的完整性”为基本原则的宏观理念.  相似文献   
70.
AimsApproximately 70% of Americans with diabetes have used complementary and alternative medicine (CAM) in the past year. Healthcare providers often receive minimal training on these therapies and subsequently rely on clinical practice guidelines (CPGs) to supplement their knowledge about the safe and effective use of CAM for the treatment/management of type 2 diabetes mellitus (T2DM). The purpose of this systematic review is to determine the quantity and assess the quality of CAM recommendations in CPGs for the treatment and/or management of T2DM.Data synthesisMEDLINE, EMBASE, and CINAHL were systematically searched from 2009 to 2020, in addition to the Guidelines International Network and the National Center for Complementary and Integrative Health websites. CPGs containing treatment and/or management recommendations for T2DM were eligible; those with CAM recommendations were quality-assessed with the AGREE II instrument twice, once for the overall CPG and once for the CAM sections. Twenty-seven CPGs were deemed eligible, of which 7 made CAM recommendations. Mean scaled domain percentages were (overall, CAM): scope and purpose (89.7%, 79.8%), clarity of presentation (85.7%, 48.4%), stakeholder involvement (67.9%, 28.2%), applicability (54.8%, 20.2%), rigour of development (49.7%, 35.7%), and editorial independence (44.1%, 44.1%).ConclusionsQuality varied within and across CPGs; domain scores across CAM sections generally scored lower than the overall CPG. Given that CAM therapies for T2DM are only represented in one-quarter of eligible CPGs and are of lower quality, a knowledge gap exists for healthcare providers who seek evidence-based information on this topic in order to effectively counsel inquiring patients.  相似文献   
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