The Myelodepletive Phenotype in Myelofibrosis: Clinical Relevance and Therapeutic Implication

Myelofibrosis (MF) is a BCR-ABL1⁻ myeloproliferative neoplasm that arises from hematopoietic stem and progenitor cells frequently harboring a somatic driver mutation in 1 of 3 genes: JAK2, CALR, or MPL. The pathologic features of this hematologic malignancy include myeloproliferation, diffuse bone marrow fibrosis, and overactivation of the JAK-STAT pathway, resulting in enhanced inflammatory cytokine release. The common clinical manifestations of MF include systemic symptoms, abnormal peripheral blood count levels, and splenomegaly. However, it has become increasingly appreciated that significant clinical heterogeneity exists among patients with MF. Two distinct MF clinical phenotypes include the myeloproliferative and myelodepletive phenotype, with peripheral blood counts being the main discerning feature. Patients with the myeloproliferative phenotype will present with elevated peripheral blood counts and often experience significant constitutional symptoms and progressive splenomegaly. In contrast, patients with the myelodepletive phenotype will have low peripheral blood counts and will frequently require transfusion support. Current frontline therapies for MF, include ruxolitinib and fedratinib, which can exacerbate cytopenias and thereby pose an impediment to effective treatment of the myelodepletive patient. The present review discusses the clinical and prognostic implications of the myelodepletive phenotype and the therapeutic options and limitations for this subset of patients, representing an unmet clinical need.     

Management recommendations for patients with chronic kidney disease during the novel coronavirus disease 2019 (COVID-19) epidemic

COVID-19 has become a pandemic and it has already spread to at least 171 countries/regions. Chronic kidney disease (CKD) is a global public health problem with a total of approximately 850 million patients with CKD worldwide and 119.5 million in China. Severe COVID-19 infection may damage the kidney and cause acute tubular necrosis, leading to proteinuria, hematuria and elevated serum creatinine. Since the SARS-CoV-2 enters the cells by binding to the angiotensin-converting enzyme 2 receptor, some doctors question its ability to increase the risk and severity of developing COVID-19. Neither clinical data nor basic scientific evidence supports this assumption. Therefore, patients who take angiotensin-converting enzyme inhibitor or angiotensin receptor blocker are not advised to change their therapy. Patients with CKD are generally the elderly population suffering from multiple comorbidities. Moreover, some patients with CKD might need to take glucocorticoids and immunosuppressants. Dialysis patients are recurrently exposed to a possible contaminated environment because their routine treatment usually requires three dialysis sessions per week. Considering all the above reasons, patients with CKD are more vulnerable to COVID-19 than the general population. The development of COVID-19 may worsen the impaired kidney function and further lead to rapid deterioration of kidney function and even death. Strict comprehensive protocols should be followed to prevent the spread of COVID-19 among patients with CKD. In this review, we provide some practical management recommendations for health care providers, patients with CKD, dialysis patients and dialysis facilities.     

急诊治疗重症心力衰竭的方案及疗效探析

目的探讨急诊治疗重症心力衰竭的方案及疗效。方法选择本院80例2016年2月-2019年2月重症心力衰竭患者。随机分组,常规治疗组采取常规方法治疗,急诊治疗组则采取全面急诊治疗。比较两组重症心力衰竭疗效;重症心力衰竭症状改善的时间、心功能改善两级的时间;治疗前后患者舒张压、平均心率以及左心射血分数;不良反应。结果急诊治疗组重症心力衰竭疗效、重症心力衰竭症状改善的时间、心功能改善两级的时间、舒张压、平均心率以及左心射血分数相比较常规治疗组更好,P<0.05。两组未见严重不良反应,P>0.05。结论全面急诊治疗重症心力衰竭可获得较好预后,可有效改善患者的心功能,缓解症状,且安全性高,无明显不良反应。     

慢性鼻窦炎指南的评价与内容分析

背景　慢性鼻窦炎是耳鼻喉科高发疾病，对患者造成严重的影响和经济负担，但目前国内外慢性鼻窦炎诊疗指南推荐意见存在差异。目的　对慢性鼻窦炎诊疗指南进行质量评价并分析其治疗意见，为指南制定和推荐意见的采纳提供建议。方法　于2019年2月检索中英文数据库和各专业指南网站获取相关文献，同时追踪参考文献，筛选适用于青少年及成年人的推荐意见中包含对慢性鼻窦炎的治疗意见的指南。检索时间为建库至检索日期。使用临床指南研究与评估系统Ⅱ（AGREE Ⅱ）和卫生保健实践指南报告清单（RIGHT）对纳入指南的方法学质量和报告质量进行评价，并绘制气泡图和思维导图，对比分析关于慢性鼻窦炎治疗的推荐意见。结果　最终纳入8部指南，AGREE Ⅱ评价总分平均为48.76%（30.90%~73.09%），仅2部指南强烈推荐使用（得分>60%），其余6部需修订后推荐。RIGHT条目总体报告率为34.29%~65.71%，其中评审和质量控制方面报告率均为0。指南推荐一致的治疗方法包括皮质类固醇、鼻腔盐水冲洗和手术治疗，以及过敏者口服抗组胺药，可选用的措施包括细菌溶解物、黏痰溶解药、质子泵抑制剂、植物疗法、辣椒素、亮氨酸拮抗剂、鼻用呋塞米、木糖醇盐水冲洗液、次氯酸钠盐水冲洗液、含婴儿香波的盐水冲洗液，尚无统一观点的治疗措施包括抗生素、减充血剂、白三烯受体拮抗剂、IgE拮抗剂及阿司匹林加重呼吸系统疾病患者阿司匹林脱敏治疗。结论　现有慢性鼻窦炎诊疗指南制定方法及其报告质量需提高，建议纳入患者的偏好、使用统一的评价工具和考虑运用性。推荐意见有冲突时，建议参考制定方法更为严谨的指南。     

综合评估慢性阻塞性肺疾病稳定期中医证型与疾病程度及预后的相关性

目的:运用综合评估法分析慢性阻塞性肺疾病稳定期中医证型与病情程度及预后的相关性。方法:将符合纳入标准的200例患者分为肺气虚、肺脾气虚、肺肾气虚及肺肾气阴两虚四组,观察1年前后患者肺功能、mMRC分级、CAT评分,并进行数据整理及统计分析。结果:肺肾气虚证、肺肾气阴两虚证患者较肺气虚证、肺脾气虚证患者肺功能分级重且FEV1下降更明显、出现呼吸困难症状重且程度进展快、对生活质量影响较大且影响增加更显著,但肺气虚证与肺脾气虚证、肺肾气虚证与肺肾气阴两虚证之间无明显差异。结论:慢性阻塞性肺疾病稳定期中医证型与疾病程度及预后存在相关性。     

Impact of pharmaceutical price controls on the cost of cardiovascular drugs: does essentiality matter?

ABSTRACT

Background

With the goal of improving the affordability of medicines, governments across the globe have instituted various forms of price controls. Since 2013, India has been regulating the prices of drugs included in its national list of essential medicines (NLEM). Here we evaluate the cost variations among available cardiovascular drugs and perform cost-analysis comparing essential and non-essential drugs.     

儿童慢性腹泻高营养风险情况及对预后的影响

目的 探讨儿童慢性腹泻高营养风险情况及对预后的影响。方法 选择2019年1～12月在我院诊断治疗的慢性腹泻患儿100例为研究对象,进行营养风险筛查,分析高营养风险患儿与非高营养风险患儿临床特征以及预后情况,分析性别、年龄、病因对慢性腹泻患儿高营养风险的影响。结果 (1)100例患儿24例患儿STAMP评分≥4分,为高营养风险组,占24.0%。(2)7～14岁患儿高营养风险发生率显著高于其他年龄段患儿(P0.05);炎症性肠病患儿高营养风险发生率显著其他病因的患儿(P0.05)。(3)炎症性肠病是慢性腹泻患儿发生高营养风险的独立危险因素(P0.05)。(4)高营养风险组白蛋白、前白蛋白水平显著低于非高营养风险组,差异有统计学意义(P0.05)。两组Hb水平比较差异无统计学意义(P0.05)。(5)非高营养风险组痊愈率显著高于高营养风险组,医院感染率显著低于高营养风险组,住院时间显著短于高营养风险组,差异均有统计学意义(P0.05)。结论 儿童慢性腹泻高营养风险发生率相对较高,病因会影响患儿高营养风险的发生,而高营养风险影响患儿的预后,延长住院时间。     

优化新生脉散方治疗心力衰竭的网络药理学机制分析

[目的] 运用网络药理学方法分析优化新生脉散方治疗慢性心力衰竭（CHF）的作用机制。[方法] 基于中药系统药理数据库和分析平台（TCMSP）、BATMAN-TCM及UniProt数据库查询优化新生脉散方药物的主要活性成分与相应靶点，利用GeneCards数据库检索CHF相关靶点，通过OmicShare网站分析并结合Cytoscape-v3.6.0软件构建优化新生脉散方中药-化合物-靶点-疾病网络图，使用DAVID数据库对潜在靶点进行基因功能（GO）及京都基因和基因组百科全书（KEGG）通路富集分析。[结果] 根据类药性（DL）、口服吸收利用度（OB）、参数评分（Scorecutoff）筛选出与CHF相关的潜在化合物85种及靶点89个，其中，化合物有quercetin、luteolin、kaempferol等，靶点包括SCN5A、ADRB2、NOS2等。功能富集分析涉及生物过程、分子功能、细胞组分的GO条目共497个，主要参与调控细胞凋亡、增殖及缺氧、炎症反应等方面，KEGG通路富集得到139条，主要与缺氧诱导因子-1（HIF-1）、磷脂酰肌醇3激酶/蛋白激酶B（PI3K-Akt）、肿瘤坏死因子（TNF）信号通路等有关。[结论] 优化新生脉散方治疗CHF具有多成分、多靶点、多途径的作用，为进一步研究优化新生脉散方治疗CHF的潜在复杂机制提供参考方向。