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DICER1 mutations in twelve Chinese patients with pleuropulmonary blastoma

Science China(Life Sciences) 2017年第7期60卷 714-720页

作者： Siyu Cai Xisi Wang Wen Zhao libing fu Xiaoli Ma Xiaoxia Peng Center for Clinical Epidemiology & Evidence-based Medicine Beijing Children's Hospital Capital Medical University National Center for Children's Health Beijing 100045 Chin Hematology Oncology Center Beijing Children's Hospital Capital Medical University Beijing 100045 China Department of Pathology Beijing Children's Hospital Capital Medical University Beijing 100045 China Beijing Key Laboratory of Pediatric Hematology Oncology Beijing 100045 China Key Laboratory of Major Diseases in Children Ministry of Education Beijng 100045 China

Our aim is to examine the impact of DICER1 mutations on the pathogenesis of pleuropulmonary blastoma(PPB) by evaluating the mutation frequency and investigating the family history of Chinese patients with PPB. The family histories of 12 children with PPB recruited consecutively were surveyed. Blood samples from patients and their first-degree relatives were tested for DICER1 mutations. Whole-genome sequencing of blood samples and formalin-fixed and paraffin-embedded(FFPE) tumor tissue was performed in one family with twins. Twelve patients with PPB included six type II and six type III cases. Seven of the12 patients harbored DICER1 mutations, six of which were frameshift or nonsense mutations. Another case carried a germline DICER1 mutation affecting the splice site. FFPE sample had a nonsense mutation in TDG and missense mutations in *** addition, two cases with DICER1 mutations were found to have lung cysts preceding the diagnosis of PPB. furthermore, one patient had a family history remarkable for thyroid diseases. Our results indicate that the germline mutation frequency in Chinese patients with PPB is similar to the ones reported for patients from USA, UK, and Japan. Moreover, our study strongly suggests that investigating the family history and detecting germline DICER1 mutations might be of benefit to increasing awareness and improving the accuracy of the differential diagnosis of PPB from non-malignant lung cysts.

关键词： China DICER1 mutation pleuropulmonary blastoma

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Temperature distribution of fluids in a two-section two-phase closed thermosyphon wellbore

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Petroleum Science 2014年第2期11卷 287-292页

作者： Zhang Yufeng Zhao Lun Fan Zifei Wu Xiaodong fu libing Xu Bifeng Kong Fanshun Jiang Shengdong Research Institute of Petroleum Exploration & Development MOE Key Laboratory of Petroleum Engineering China University of Petroleum School of Energy Resources China University of Geosciences Exploration and Development Research Institute Petrochina Daqing Oilfield Company Northeast Petroleum University

Compared with a conventional single section two-phase closed thermosyphon （TPCT） wellbore, a two-section TPCT wellbore has better heat transfer performance, which may improve the temperature distribution of fluid in wellbores, increase the temperature of fluid in wellheads and even more effectively reduce the failure rate of conventional TPCT wellbores. Heat transfer performance of two-section TPCT wellbores is affected by working medium, combination mode and oil flow rate. Different working media are introduced into the upper and lower TPCTs, which may achieve a better match between the working medium and the temperature field in the wellbores. Interdependence exists between the combination mode and the flow rate of the oil, which affects the heat transfer performance of a two-section TPCT wellbore. The experimental results show that a two-section TPCT wellbore, with equal upper and lower TPCTs respectively filled with Freon and methanol, has the best heat transfer performance when the oil flow rate is 200 L/h.

关键词： Two-section two-phase closed thermosyphon temperature distribution combination mode,heat transfer performance

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Detection of FOXO1 break-apart status by fluorescence in situ hybridization in atypical alveolar rhabdomyosarcoma

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Science China(Life Sciences) 2017年第7期60卷 721-728页

作者： libing fu Yaqiong Jin Chao Jia Jie Zhang Jun Tai Hongbin Li Feng Chen Jin Shi Yongli Guo Xin Ni Lejian He Department of Pathology Beijing Children's Hospital Capital Medical University National Center for Children's Health Beijing 100045 China Beijing Key Laboratory for Pediatric Diseases of Otolaryngology Head and Neck Surgery MOE Key Laboratory of Major Diseases in Children Beijing Pediatric Research Institute Beijing Children's Hospital Capital Medical University National Center for Children's Health Beijing 100045 China Biobank for Clinical Data and Samples in Pediatric Beijing Pediatric Research Institute Beijing Children's Hospital Capital Medical University National Center for Children's Health Belting 100045 China Department of Otolaryngology Head and Neck Surgery Beijing Children's Hospital Capital Medical University National Center for Children's Health Beijing 100045 China

The morphologies of alveolar rhabdomyosarcoma(ARMS) are various. Some cases entirely lack an alveolar pattern and instead display a histological pattern that overlaps with embryonal rhabdomyosarcoma(ERMS). The method of pathological diagnosis of ARMS and ERMS has been updated in the 4th edition of the World Health Organization's guidelines for classification of skeletal muscle tumors. Under the new guidelines, there is still no molecular test to distinguish between these two subtypes of rhabdomyosarcoma(RMS). In the present study, we applied fluorescent in situ hybridization(FISH) and found that the Forkhead box O1(FOXO1) gene broke apart, amplified, and displayed an aneuploid signal that was related to the RMS pathological *** from the fact that FOXO1 break-apart and its amplification were correlated with atypical ARMS, aneuploidies were usually found in atypical ERMS. In conclusion, our results detail a potential biomarker to improve the accuracy of pathological diagnosis by discriminating between atypical ARMS and atypical ERMS.

关键词： atypical alveolar rhabdomyosarcoma FOXO1 fluorescence in situ hybridization

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Biallelic inactivation of SDHA results in comorbidity of pediatric recurrent neuroblastoma and gastric stromal tumor

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Genes & Diseases 2024年第6期11卷 99-102页

作者： Shen Yang Shixuan Zhang Wen Zhao libing fu Li Zhang Chan Hon Chui Ruolan Guo Yan Su Dayan Sun Huanmin Wang Department of Oncology Surgery Beijing Children's HospitalCapital Medical UniversityNational Center for Children's HealthBeijing 100045China State Key Laboratory of Genetic Engineering Collaborative Innovation Center for Genetics and DevelopmentSchool of Life Sciences and Human Phenome InstituteFudan UniversityShanghai 200438China Hematology Oncology Center Beijing Children's HospitalCapital Medical UniversityNational Center for Children's HealthBeijing100045China Department of Pathology Beijing Children's HospitalCapital Medical UniversityNational Center for Children's HealthBeijing 100045China Shanghai Institute of Precision Medicine Shanghai Jiao Tong University School of MedicineShanghai 200011China Surgery Centre for Children Mount Elizabeth Medical Centre228510Singapore Beijing Key Laboratory for Genetics of Birth Defects Beijing Pediatric Research instituteMOE Key Laboratory of Major Diseases in ChildrenGenetics and Birth Defects Control CenterBeijing Children's HospitalCapital Medical UniversityNational Center for Children's HealthBeijing 100045China Department of Neonatal Surgery Beijing Children's HospitalCapital Medical UniversityNational Center for Children's HealthBeijing 100045China MOE Key Laboratory of Major Diseases in Children Beijing Children's HospitalCapital Medical UniversityNational Centerfor Children's HealthBeijing 100045China

Neuroblastoma(NB)is a common pediatric extracranial solid tumor that exhibits varied characteristics,clinical features,and prognosis.1 Totally 1%-2%of cases show familial history with genetic links like ALK,PHOX2B mut... 详细信息

关键词： pediatric neoplasms neuroblastoma

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Correlation between BRAF^(V600E) mutation and clinicopathological features in pediatric papillary thyroid carcinoma

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Science China(Life Sciences) 2017年第7期60卷 729-738页

作者： Jiangqiao Geng Huanmin Wang Yuanhu Liu Jun Tai Yaqiong Jin Jie Zhang Lejian He libing fu Hong Qin Yingluan Song Jinzhu Su Aiying Zhang Xin Wen Yongli Guo Xin Ni Otolaryngology Head and Neck Surgery Beijing Children's Hospital Capital Medical University National Center for Children 's Health Beijing 100045 Chin Otolaryngology Children's Hospital of Hebei Province Shijiazhuang 050031 China Department of Surgical Oncology Beijing Children's Hospital Capital Medical University National Center for Children's Health Beijing 100045 China Beijing Key Laboratory for Pediatric Diseases of Otolaryngology Head and Neck Surgery Beo'ing Pediatric Research Institute Beijing Children 's Hospital Capital Medical University National Center for Children's Health Beijing 100045 China Department of Pathology Beijing Children's Hospital Capital Medical University National Center for Children's Health Beijing 100045 China

In adults, the presence of the BRAF^(V600E) mutation in papillary thyroid cancer(PTC) has been demonstrated to be strongly associated with aggressive cancer-cell characteristics and poor patient prognosis. In contrast, the frequency of this mutation in pediatric PTC has undergone limited study, and the few available estimates range from 0 to 63%. furthermore, the role of the BRAF^(V600E) mutation in pediatric PTC is controversial; thus, the present study aimed to investigate the prevalence and role of the BRAF^(V600E) mutation in48 pediatric patients with PTC, aged 3–13 years. Of these patients, 41 were diagnosed with classic PTC, five were found to have a follicular variant of PTC, and two to exhibit a diffuse sclerosing PTC variant. The BRAF^(V600E) mutation was identified to be present in 35.4% of the 48 analyzed patients, and in 41.5% of the patients diagnosed with classical PTC. furthermore, the presence of the BRAF^(V600E) mutation was found to be associated with a patient age at diagnosis of less than ten years(P=0.011), the performance of a thyroidectomy(P=0.03), exhibited tumor multifocality(P=0.02) and/or extra-thyroidal invasion(P=0.003), and both a low MACIS(Metastases, Age, Completeness of resection, Invasion, Size)(P=0.036) and AMES(Age, Metastasis, Extent of tumor,Size)(P=0.001)score. Together, these data suggest that the presence of the BRAF^(V600E) mutation may be negatively correlated with partial aggressive clinicopathological features of pediatric PTC.

关键词： pediatric papillary thyroid cancer BRAF^(V600E) mutation clinicopathological characteristics

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桩筏基础荷载分担的分析与建议

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四川建筑科学研究 2011年第5期37卷 149-151页

作者：付利兵中国建筑西南设计研究院有限公司四川成都610042

分析了影响桩筏基础荷载分担的诸多因素,引入有限元结合统计的简化公式,结合影响因素分析了此公式的合理性,并指出了该公式的缺陷及改进的思路。

关键词：桩筏基础荷载分担简化公式

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Hepatoblastoma with neonatal necrotizing enterocolitis:Two case reports

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Cancer Innovation 2023年第6期2卷 532-536页

作者： Sidou He Xisi Wang Chao Duan Wen Zhao Chiyi Jiang Shihan Zhang Binglin Jian Wei Yang Tong Yu libing fu Huanmin Wang Xiaoli Ma Medical Oncology Department Pediatric Oncology CenterBeijing Children's HospitalCapital Medical UniversityNational Center for Children's HealthBeijingChina Beijing Key Laboratory of Pediatric Hematology Oncology National Key Clinical Discipline of Pediatric OncologyKey Laboratory of Major Diseases in ChildrenMinistry of EducationBeijingChina Surgical Oncology Department Beijing Children's HospitalCapital Medical UniversityNational Center for Children's HealthBeijingChina Imaging Center Department Beijing Children's HospitalCapital Medical UniversityNational Center for Children's HealthBeijingChina Pathology Department Beijing Children's HospitalCapital Medical UniversityNational Center for Children's HealthBeijingChina

We report two children with hepatoblastoma(HB)with a history of neonatal necrotizing enterocolitis(NEC).Case 1 was diagnosed with HB at 5 months of *** enlargement was found during the NEC operation at 3 months of age and then was clinically diagnosed by *** six chemotherapy courses,a partial hepatectomy was *** months after ceasing the chemotherapy,a chest computed tomography scan suggested that distant metastasis of the tumor should be considered,and the lesion was ***,9 months after the operation,alpha-fetoprotein concentrations were increased,and abdominal imaging showed a recurrence of the tumor in situ,resulting in a *** 2 was diagnosed with NEC shortly after birth and underwent an intestinal resection and anastomosis 1 month *** was diagnosed with HB at 3 years of *** was performed after five courses of *** was stopped after 10 courses,and alpha-fetoprotein concentrations were *** present,both children have survived and are in a healthy *** should be aware of the possibility of HB and a history of NEC in *** birth and low birth weight are common factors leading to the pathogenesis of HB and *** association between these two diseases requires further study。

关键词： hepatoblastoma LGR5 low birth weight neonatal necrotizing enterocolitis premature delivery

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幂函数递减模型的建立、对比与应用

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油气地质与采收率 2019年第6期26卷 87-91页

作者：陈元千傅礼兵中国石油勘探开发研究院

Arps基于矿场油井产量递减数据的统计分析和理论研究,提出了指数、双曲线和调和三种经典的递减模型,受到世人的高度重视,至今被广泛地应用于全球油气工业的产量和可采储量的评价。应当指出,Arps的指数递减是递减率为常数且产量递减较快... 详细信息

Arps基于矿场油井产量递减数据的统计分析和理论研究,提出了指数、双曲线和调和三种经典的递减模型,受到世人的高度重视,至今被广泛地应用于全球油气工业的产量和可采储量的评价。应当指出,Arps的指数递减是递减率为常数且产量递减较快的一种递减模型。由于其预测的指标比较稳妥,被上市评估公司普遍采用。Arps的双曲线递减和调和递减是两种产量递减较慢的模型,在实际中应用得并不多。陈元千等于2015年建立的线性递减模型,仅适用于产量随时间呈直线下降,而且递减率随时间的增加而增加。本文建立的幂函数递减模型,它的递减率随时间的增加而降低。根据Arps提出的递减指数n划分的不同递减模型:n=-1时为线性递减,n=0时为指数递减,0

关键词：幂函数递减预测模型产量累积产量经济可采储量采出程度递减率

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热流道辅助长筒薄壁形锌合金件压铸成型研究

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材料科学与工艺 2016年第4期24卷 25-32页

作者：戴伟付利兵刘佳张琦材料成形与模具技术国家重点实验室(华中科技大学) 武汉430074 武汉轻工大学机械工程学院武汉430023

ZA8锌合金以其卓越的耐冲击性能和尺寸稳定性,在精密电子类产品中得到广泛的应用.但这种合金材料的热流动性能较差,在其压铸成型中会造成很多的压铸缺陷.为减少薄壁ZA8锌合金铸件压铸生产中出现的流纹、缩松、气孔、起泡等缺陷,本文在... 详细信息

ZA8锌合金以其卓越的耐冲击性能和尺寸稳定性,在精密电子类产品中得到广泛的应用.但这种合金材料的热流动性能较差,在其压铸成型中会造成很多的压铸缺陷.为减少薄壁ZA8锌合金铸件压铸生产中出现的流纹、缩松、气孔、起泡等缺陷,本文在采用传统的锌合金压铸流道实验的基础上,调节各种工艺参数,对其产生的压铸缺陷进行了统计分析.分析结果显示,流道结构是该铸件缺陷产生的主要原因,改用热流道辅助压铸成型.重新统计后发现,热流道能有效地减少铸件出现的各种缺陷,且材料利用率可以从30.6%提高到41.9%.在对比压铸传统流道和热流道特点的基础上,给出了热流道的设计和安装结构,详细阐述了压铸热流道技术的原理,分析了采用压铸热流道技术所带来的经济效益.研究得出,锌合金压铸热流道相比于传统的流道,在生产中具有加温时间快、产品成型周期短、温度压力损失小、材料利用率高、铸件缺陷低等优点.

关键词： ZA8锌合金压力铸造浇道缺陷热流道

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评过剩吸附量和绝对吸附量关系式的不正确性

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油气地质与采收率 2020年第2期27卷 1-6页

作者：陈元千傅礼兵徐佳倩中国石油勘探开发研究院北京100083

页岩吸附气和煤层吸附气都是重要的非常规天然气资源。该资源的评价依赖于在不同压力下等温累积吸附量的计算和等温饱和吸附量的确定。不同压力下的等温累积吸附量,需要通过吸附仪的测试资料,利用有关的计算方法求得。基于1976年Adamson... 详细信息

页岩吸附气和煤层吸附气都是重要的非常规天然气资源。该资源的评价依赖于在不同压力下等温累积吸附量的计算和等温饱和吸附量的确定。不同压力下的等温累积吸附量,需要通过吸附仪的测试资料,利用有关的计算方法求得。基于1976年Adamson(阿达姆森)提出的固相对气相吸附质量的关系式是不确定的,因此,1998年Donohue和Aranovich引用Adamson的吸附关系式,并提出过剩吸附量和绝对吸附量及其关系式也是不正确的。周理等于2000年发表的文章,确认和引用了Donohue和Aranovich过剩吸附量和绝对吸附量及其关系式同样是不正确的。在2015至2018年间,俞凌杰等沿用周理等的过剩吸附量和绝对吸附量关系式,发表了多篇论文。通过本文的研究,认为过剩吸附量和绝对吸附量是两个缺少物理定义和使用价值的伪名词,两者之间的关系式也是不正确的。利用过剩吸附量和绝对吸附量的关系式根本得不到等温吸附量、等温累积吸附量和等温饱和吸附量。

关键词：页岩煤层吸附气过剩吸附量绝对吸附量关系式不正确性

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