Metastatic adult-type non-rhabdomyosarcoma soft tissue sarcomas in children and adolescents: A cohort study from the European paediatric Soft tissue sarcoma Study Group

Background

Limited data exist on the clinical behavior of pediatric non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) with distant metastases at onset, and a clear standard of care has not yet been defined.

Methods

This cohort study reports on pediatric adult-type metastatic NRSTS enrolled in two concurrent prospective European studies, i.e., the randomized BERNIE study and the single-arm MTS 2008 study developed by the European paediatric Soft tissue sarcoma Study Group. Treatment programs were originally designed for patients with metastatic rhabdomyosarcoma, i.e., nine courses of multidrug chemotherapy (with or without bevacizumab in the BERNIE study), followed by 12 cycles of maintenance therapy, whereas radiotherapy and/or surgery (on primary tumor and/or metastases) were delayed until after seven courses of chemotherapy had been administered.

Results

The study included 61 patients <21 years old treated from July 2008 to December 2016. The lung was the site of metastases in 75% of the cases. All patients received multi-agent chemotherapy, 44% had local therapy to primary tumor, and 18% had treatment of metastases. Median time to progression/relapse was 6 months. A high rate of tumor progression was observed during the initial part of the chemotherapy program. With a median follow-up of 41.5 months (range, 2–111 months), 3-year event-free survival and overall survival were 15.4% (95% confidence interval [CI], 7.6–25.7) and 34.9% (95% CI, 22.7–47.5), respectively. There were no statistically significant differences in outcome depending on the type of treatment administered.

Conclusions

The study confirmed the overall poor outcome for patients with metastatic NRSTS, whose treatment remains a challenge.

Plain Language Summary

• Pediatric non-rhabdomyosarcoma soft tissue sarcomas form a heterogeneous group of rare tumors.
• Although recent international studies have defined the standard of care for patients with localized disease, limited data are available on the clinical behavior of patients with distant metastases.
• This study on 61 metastatic cases treated on two prospective European protocols confirms that the chances of survival of such patients are often dismal and a standard treatment is still lacking.

     

厦门市社区老年慢性病共病与不良生活方式共存现状研究

目的 调查社区老年人常见的14种慢性病共病情况及不良生活方式共存现象。 方法 采用方便抽样的方法抽取厦门市社区906例老年人群为研究对象,使用自设问卷调查研究对象的慢病共存及不良方式共存现象,使用检验和多因素logistic回归方法进行分析。结果 共纳入906例老年人,慢性病患病率为79.5%,老年人慢性病共病患病率为58.9%。主要的不良生活方式是BMI异常(46.8%)、饮酒(40.5%)、睡眠时间少(37.9%)、缺乏锻炼(35.2%)、吸烟(32.8%)等。多因素logistic回归分析显示,女性(OR=2.232,95%CI:1.474～3.380,P＜0.001)、高龄(OR=2.038,95%CI:1.234～3.365,P=0.001)、有慢性病家族史(OR=2.854,95%CI:1.943～4.194,P＜0.001)、肥胖(OR=2.571,95%CI:1.096～6.033,P=0.030)、饮酒(OR=3.582,95%CI:2.531～5.071,P＜0.001)、吸烟(OR=1.789,95%CI:1.172～2.732,P=0.007)、嗜盐(OR=1.818,95%CI:1.170～2.823,P=0.008)、嗜油(OR=2.023,95%CI:1.153～3.550,P=0.010)、睡眠质量差(OR=2.091,95%CI:1.360～3.215,P=0.001)的老年人,慢性病共病的比例高。 结论 厦门市社区老年人慢性病共病和不良生活方式共存现象严重。肥胖、饮酒、吸烟、嗜盐、嗜油、睡眠质量差等行为生活方式是慢性病重要的可干预因素,社区工作者应提高社区居民对健康生活方式重要性的认识,促使其主动改变不良生活方式并长期坚持健康的生活方式,以降低其慢性病的发病风险,减少其伤残程度,提高生活质量。     

英语学界对“母乳不足”现象的认知进化

“母乳最好”的宣传话语在目前育龄人群中几乎耳熟能详,但很多信奉科学育儿、希望母乳喂养的都市女性却发现母乳喂养困难重重,其中遭遇“母乳不足”的案例较为多见。回顾历史发现,“母乳不足”的现象全球普遍存在,这个话题在20世纪80年代的英语学术圈曾引起热议。经过十余年讨论后,学者们基本认同大部分遭遇母乳不足的女性并不是真正的“母乳不足”,而是由于种种社会文化因素干扰造成母乳喂养失败的这一结论,其中产后初期的奶瓶介入是重要原因。术语也由insufficient milk syndrome变为perceived insufficient milk,以此来强调“母乳不足”不是一种病理现象,而更多是一种文化感知。     

江苏省专科护士工作投入现状及影响因素调查分析

目的 了解江苏省省级专科护士工作投入现状及影响因素，为针对性管理提供参考。方法 采用一般资料调查问卷与专科护士工作投入量表对2 472名省级专科护士进行调查分析。结果 专科护士工作投入总均分为4.06±0.46;多元线性回归分析显示，性别、聘任方式、专科类别、工作类型、出任专科护理门诊、论文发表是专科护士工作投入的影响因素(P<0.05,P<0.01)。结论 专科护士工作投入水平较高，其影响因素较多，护理管理者应关注专科护士专业开展现况，采取针对性措施支持专科护理的有效开展，从而进一步提高专科护士的工作投入水平。     

家长对小学生近视的知信行分析

目的 了解保定市小学生家长关于小学生近视知信行的相关现状及影响因素,制定有效干预措施,为预防儿童青少年近视提供有力依据。方法 运用分层整群随机抽样的方法,抽取保定市某小学一年级至六年级766名学生家长进行问卷调查。结果 小学生家长用眼卫生相关知识总正确率为64.90%。家长年龄越大、文化程度越高以及自身近视的家长掌握小学生近视的相关知识越多。家长年龄越大,督导孩子健康用眼的行为越易发生(P=0.027,OR=0.169)。结论 应多渠道对学生家长进行健康教育,为控制和预防小学生近视的发生与发展构建以家庭为主的第一道防线。     

Perinatal and familial risk factors for soft tissue sarcomas in childhood through young adulthood: A population-based assessment in 4 million live births

Perinatal factors have been associated with soft tissue sarcomas (STS) in case-control studies. However, (i) the contributions of factors including fetal growth remain unknown, ( ii ) these factors have not been examined in cohort studies and (iii) few assessments have evaluated risk in specific STS subtypes. We sought to identify the role of perinatal and familial factors on the risk of STS in a large population-based birth cohort. We identified 4,023,436 individuals in the Swedish Birth Registry born during 1973–2012. Subjects were linked to the Swedish Cancer Registry, where incident STS cases were identified. We evaluated perinatal and familial factors obtained from Statistics Sweden, including fetal growth, gestational age, and presence of a congenital malformation. Poisson regression was used to estimate incidence rate ratios (IRRs) and 95% confidence intervals (CIs) for associations between perinatal factors and STS overall, as well as by common subtypes. There were 673 individuals diagnosed with STS in 77.5 million person-years of follow-up. Having a congenital malformation was associated with STS (IRR = 1.70, 95% CI: 1.23–2.35). This association was stronger (IRR = 2.90, 95% CI: 1.25–6.71) in recent years (2000–2012). Low fetal growth was also associated with STS during the same time period (IRR = 1.86, 95% CI: 1.05–3.29). Being born preterm was associated with rhabdomyosarcoma (IRR = 1.74, 95% CI: 1.08–2.79). In our cohort study, those with congenital malformations and other adverse birth outcomes were more likely to develop a STS compared to their unaffected contemporaries. These associations may point to disrupted developmental pathways and genetic factors influencing the risk of STS.     

加味黄芪桂枝五物汤联合醒脑开窍针刺法治疗脑卒中后肩手综合征的临床观察

目的:观察加味黄芪桂枝五物汤联合醒脑开窍针刺法治疗脑卒中后肩手综合征(SHS)的临床疗效及对神经源性炎症介质和血液流变学指标的影响。方法:将148例患者随机按数字表法分为对照组和观察组各74例。两组口服双氯芬酸钠缓释片,75 min/次,1次/d,连续2～4周;肿胀明显,口服醋酸泼尼松片,10 min/次,1次/d,连续1～2周。并采用醒脑开窍针刺法,1次/d,6次/周;对照组口服脑心通胶囊,4粒/次,3次/d,观察组内服加味黄芪桂枝五物汤,1剂/d。两组疗程均为连续治疗4周。进行对治疗前后肩手综合征评估量表(SHSS)评分,记录疼痛、肿胀消失时间;进行治疗前后Fugl-Meyer功能量表上肢部分评分(U-FMA),日常生活活动能力(ADL)评分和气虚血瘀证评分;检测治疗前后降钙素基因相关肽(CGRP),P物质(SP),缓激肽(BK)水平和血液流变学指标。结果:观察组患者的临床疗效优于对照组(Z=2. 106,P0. 05);观察组SHSS量表的感觉、自主神经、运动3个维度评分和SHSS总分均低于对照组(P0. 01);观察组疼痛、肿胀消失时间均短于对照组(P0. 01);观察组患者U-FMA,ADL评分均高于对照组(P0. 01),气虚血瘀证评分低于对照组(P0. 01);观察组CGRP水平高于对照组(P0. 01),SP和BK水平均低于对照组(P0. 01);观察组的全血黏度(高切、低切)、血浆黏度、纤维蛋白原和血小板聚集率等均低于对照组(P0. 05)。结论:在西医常规治疗的基础上,内服加味黄芪桂枝五物汤配合醒脑开窍针刺疗法可减轻SHS严重程度和中医临床证候,缩短病程,改善上肢运动功能,并可抑制神经源性炎症反应,改善血液流性,提高患者的日常生活活动能力和临床疗效。     

从血浊理论浅谈动脉粥样硬化的危险因素

血浊作为全新的中医学理念,为心脑血管疾病的防治提供了新的思路与方法,动脉粥样硬化作为心脑血管疾病的病理基础,危险因素广泛,对人们的生命安全威胁极大,故从血浊理论探讨动脉粥样硬化的危险因素,初步阐释血浊与动脉粥样硬化发生、发展的关系。血浊理论是动脉粥样硬化的病理基础,从这一角度为动脉粥样硬化的防治提供了新思路、新方法。     

甲状腺未分化癌发生肺转移的危险因素分析

目的:探讨甲状腺未分化癌(anaplastic thyroid carcinoma,ATC)发生肺转移的危险因素。方法:回顾性分析2010年至2015年SEER数据库中被确诊为ATC的405例患者临床病理资料。通过对年龄、种族、性别、原发灶最大径、原发灶数量、甲状腺外组织侵犯、食管侵犯、气管侵犯、中央区淋巴结转移、颈侧区淋巴结转移10个因素进行单因素分析,进而对单因素分析中有统计学差异的指标行多因素Logistic回归分析,寻找ATC患者发生肺转移的危险因素。结果:405例ATC患者中,发生肺转移107例,未发生肺转移298例。单因素分析显示:原发灶最大径>4 cm、甲状腺外组织侵犯、颈侧区淋巴结转移是预测发生肺转移的显著性因素(均P<0.05)。多因素Logistic回归分析显示:原发灶最大径>4 cm及颈侧区淋巴结转移是ATC患者发生肺转移的独立危险因素(均P<0.05)。结论:原发灶最大径>4 cm、颈侧区淋巴结转移的ATC患者极易发生肺转移,因此,一定要认真参考病理结果,若发现存在以上肺转移危险因素,则积极采取多模式治疗方案,并缩短随访时间,行相关检查(如胸部CT扫描、PET-CT等)。     

基于网络药理学的中期诊疗方干预新型冠状病毒肺炎(COVID-19)作用机制研究

目的通过网络药理学方法研究新型冠状病毒肺炎(COVID-19)中期诊疗方(XGZQZLF)治疗COVID-19的作用机制。方法采用网络药理学方法,以中药系统药理学数据库、GeneCards数据库等为支撑,通过Cytoscape构建"药物-活性成分-靶点-疾病"网络,进一步进行基因富集分析和通路注释,研究XGZQZLF治疗COVID-19的物质基础和作用机制。结果筛选出XGZQZLF有效成分80个,通过调控蛋白激酶(AKT1)、白细胞介素6(IL-6)、丝裂原活化蛋白激酶3(MAPK3)、血管内皮生长因子A(VEGFA)、半胱氨酸蛋白酶3(CASP3)等208个肺炎相关靶点,以及主要通过调控TNF信号通路、IL-17信号通路、Th17细胞分化、缺氧诱导因子1(HIF-1)信号通路等炎症和免疫相关通路发挥治疗COVID-19的作用。结论 XGZQZLF通过多成分、多靶点、多通路协同作用发挥治疗COVID-19的作用,为XGZQZLF治疗COVID-19的生物机制研究提供数据支撑,同时为COVID-19药物研发提供参考。