双侧耳郭软骨骨化1例并文献复习

目的探讨耳郭软骨骨化的发病机制、临床表现、诊断依据、鉴别诊断、治疗方法。方法回顾性分析1例耳郭软骨骨化患者的临床资料并复习相关文献进行总结。结果耳郭软骨骨化的发病机制暂不明确,通常无明显不适症状,少数患者可伴有局部疼痛、听力下降等症状。颞骨CT可辅助诊断,最终确诊依赖于组织病理检查。症状不明显时可暂予观察,若症状严重影响了生活和工作,可予手术治疗。结论耳郭软骨骨化在临床上较为少见,目前尚无有效逆转耳郭骨化的办法。积极寻找病因,及早干预,有利于避免病情进一步发展。     

Characterizing Standardized Functional Data at Inpatient Rehabilitation Facilities

ObjectiveThe Improving Medicare Post-Acute Care Transformation Act of 2014 mandates using standardized patient functional data across post-acute settings. This study characterized similarities and differences in clinician-observed scores of self-care and transfer items for the standardized section GG functional domain and the functional independent measure (FIM) at inpatient rehabilitation facilities.DesignWe conducted secondary analyses of 2017 Uniform Data System for Medical Rehabilitation national data. Patients were assessed by clinicians on both section GG and FIM at admission and discharge. We identified 7 self-care items and 6 transfer items in section GG conceptually equivalent with FIM. Clinician-assessed scores for each pair of items were examined using score distributions, Bland-Altman plot, correlation (Pearson coefficients), and agreement (kappa and weighted kappa) analyses.Setting and ParticipantsIn all, 408,491 patients were admitted to Uniform Data System for Medical Rehabilitation-affiliated inpatient rehabilitation facilities with one of the following impairments: stroke, brain dysfunction, neurologic condition, orthopedic disorders, and debility.MeasuresSection GG and FIM.ResultsPatients were scored as more functionally independent in section GG compared with FIM, but change score distributions and score orders within impairment groups were similar. Total scores in section GG had strong positive correlations (self-care: r = 0.87 and 0.95; transfer: r = 0.82 and 0.90 at admission and discharge, respectively) with total FIM scores. Weak to moderate ranking agreements with total FIM scores were observed (self-care: kappa = 0.49 and 0.60; transfers: kappa = 0.43 and 0.52 at admission and discharge, respectively). Lower agreements were observed for less able patients at admission and for higher ability patients of their change scores.Conclusions and ImplicationsOverall, response patterns were similar in section GG and FIM across impairments. However, variations exist in score distributions and ranking agreement. Future research should examine the use of GG codes to maintain effective care, outcomes, and unbiased reimbursement across post-acute settings.     

英国癌症药物基金改革及对医保创新药支付政策的启示

目的:通过对英国癌症药物基金(Cancer Drugs Fund, CDF)的形成背景、运行机制及改革中优化管理策略进行批判借鉴研究,为中国创新药准入及管理政策的制定提供参考。方法:文献梳理、政策分析。结果:CDF在解决癌症患者创新药的可及性、可负担性方面具有积极意义,但同时存在较大争议。CDF通过不断改革完善、优化基金管理办法,在药品准入和退出机制、审批流程规范化、预算管理风险分摊协议等方面做出优化调整。结论和建议:CDF在癌症创新药快速准入方面的经验及教训值得我国批判借鉴。中国的创新药支付政策方面,建议可成立多元化筹资的癌症药物基金,建立药物准入及退出机制,关注真实世界数据在支付决策中的作用,探索合理超支分担科学管理的预算协议。     

Pseudoxanthoma elasticum-like papillary dermal elastolysis in non-exposed skin

Pseudoxanthoma elasticum-like papillary dermal elastolysis is an acquired elastic tissue disorder clinically similar to pseudoxanthoma elasticum in the absence of systemic involvement. Histopathologically, special staining of elastic fibers demonstrates a total or partial band-like loss of elastic fibers in the papillary dermis. Although ultraviolet radiation seems to be one of the main etiological factors in this entity, we report a case of pseudoxanthoma elasticum-like papillary dermal elastolysis on the neck of a woman who wore hijab.     

Trends in U.S. Ambulatory Cardiovascular Care 2013 to 2017: JACC Review Topic of the Week

The National Cardiovascular Data Registry PINNACLE (Practice Innovation and Clinical Excellence) Registry is the largest outpatient cardiovascular practice registry in the world. It tracks real-world management and quality of 4 common cardiovascular conditions: heart failure, coronary artery disease, atrial fibrillation, and hypertension. In 2013, the PINNACLE Registry contained information on 2,898,505 patients, cared for by 4,859 providers in 431 practices. By 2017, the registry contained information on 6,040,996 patients, cared for by 8,853 providers in 724 practices. During this time period, care processes for PINNACLE patients generally improved. Among patients with heart failure, combined beta-blocker and renin-angiotensin antagonist medication rates increased from 60.7% to 72.8%. Among patients with coronary artery disease, statin medication rates increased from 66% to 80.1%. Among patients with atrial fibrillation, oral anticoagulation rates increased from 52.7% to 65.2%. In contrast, blood pressure control rates among patients with hypertension were largely stable. PINNACLE data also fueled a variety of quality measurement programs and 51 peer-reviewed publications.     

观察弹性髓内钉在儿童四肢长骨骨折中的治疗效果

目的对弹性髓内钉在儿童四肢长骨骨折中的治疗效果进行观察。方法资料选自2011年11月~2012年11月我院收治的四肢长骨骨折患儿92例,通过弹性髓内钉对患儿进行治疗。结果所有患儿的治疗效果评价中优良率为96.74%,说明弹性髓内钉治疗儿童四肢长骨骨折的效果较好。结论采用弹性髓内钉对四肢长骨骨患儿进行治疗的效果较好。     

Transmission and scanning electron microscopy of cutis rhomboidalis

Cutis rhomboidalis nuchae was assessed in a 65-year-old patient. Optical microscopy showed basophilic agglomerations in the reticular dermis with decreased elastic fibers. Transmission electron microscopy showed elongated, curved and fragmented structures, and in their interior the presence of electron-dense lumps was reduced and irregular, similar to modified elastic fibers, whereas the collagen fibers had a normal aspect. Scanning electron microscopy showed deposits between the bundles of collagen, resembling pebbles or stones. These findings demonstrate that, at one stage of the disease, the collagen remains normal and the alterations are seen in the elastic tissue.     

三种统计分析方法在基因表达谱数据中的比较研究

目的比较SCAD-支持向量机、支持向量机和弹性网三种方法对基因表达谱数据的变量筛选和预测判别能力。方法根据设置的参数生成不同条件的基因表达谱模拟数据和实际数据,利用FDR、一致性错误率和ROC曲线下面积(AUC值)从三个方面评价三种方法的变量筛选和预测判别能力。结果模拟实验显示在差异变量数不变的情况下,随着差异变量间相关系数的增加,三种方法建立模型的变量筛选和预测判别能力均提高;当差异变量间相关系数不变时,随着差异变量数目的增加,SCAD-支持向量机和弹性网方法的变量筛选和预测判别能力均呈下降趋势,而支持向量机呈现提高趋势。结论 SCAD-支持向量机不仅改善了支持向量机不能直接进行变量筛选的不足同时提高了模型的精度以及判别的准确性。综合来看SCAD-支持向量机的变量筛选和预测判别能力更优,处理变量间有高度相关性的基因表达谱数据时可以获得更高的预测精度和更稳定的模型估计。