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Passive Smoking During the Periconceptional Period and Risk for Neural Tube Defects in Offspring—Five Counties,Shanxi Province,China,2010–2016

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China CDC weekly 2021年第37期3卷 778-782页

作者： Yali Zhang Jufen Liu Le Zhang Lei Jin Nicholas D E Greene Zhiwen Li Aiguo Ren institute of Reproductive and child health/Key Laboratory of Reproductive health National Health Commission of the People’s Republic of ChinaPeking UniversityBeijingChina Department of Epidemiology and Biostatistics School of Public HealthPeking UniversityBeijingChina UCL Great Ormond Street Institute of Child Health University College LondonLondonUK.

Summary What is already known about this topic?Passive smoking during pregnancy,which is prevalent in China,has been reported to be associated with an increased risk for neural tube defects(NTDs)in five counties of Shanxi Province in northern China.What is added by this report?After 4 years since the implementation of the World health Organization(WHO)Framework Convention on Tobacco Control(FCTC)in China.

关键词： China. Shanxi Period

Gene therapy and genome editing for primary immunodeficiency diseases

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Genes & Diseases 2020年第1期7卷 38-51页

作者： Zhi-Yong Zhang Adrian J.Thrasher Fang Zhang Molecular and Cellular Immunology Great Ormond Street Institute of Child HealthUniversity Colleage LondonUK Department of Immunology and Rheumatology Children’s Hospital of Chongqing Medical UniversityChina

In past two decades the gene therapy using genetic modified autologous hematopoietic stem cells(HSCs)transduced with the viral vector has become a promising alternative option for treating primary immunodeficiency diseases(PIDs).Despite of some pitfalls at early stage clinical trials,the field of gene therapy has advanced significantly in the last decade with improvements in viral vector safety,preparatory regime for manufacturing high quality virus,automated CD34 cell purification.Hence,the overall outcome from the clinical trials for the different PIDs has been very encouraging.In addition to the viral vector based gene therapy,the recent fast moving forward developments in genome editing using engineered nucleases in HSCs has provided a new promising platform for the treatment of PIDs.This review provides an overall outcome and progress in gene therapy clinical trials for SCID-X,ADA-SCID,WAS,X-CGD,and the recent developments in genome editing technology applied in HSCs for developing potential therapy,particular in the key studies for PIDs.

关键词： Adenosine deaminase deficient Chronic granulomatous disease Gene therapy Genome editing Hematopoietic progenitor stem cells Primary immunodeficiency diseases Wiskott-Aldrich syndrome X-liked severe combined immunodeficiency

Maternal mental health and well-being during the COVID-19 pandemic in Beijing, China

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World Journal of Pediatrics 2021年第3期17卷 280-289页

作者： Zhuang Wei Ming-Yue Gao Mary Fewtrell Jonathan Wells Jin-Yue Yu Department of child healthcare Beijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthBeijingChina Population Policy and Practice Research and Teaching DepartmentUCL Great Ormond Street Institute of Child Health30 Guilford StreetLondon WC1N 1EHUK

Background The aim of this study is to evaluate the impact of the coronavirus disease 2019(COVID-19)pandemic on breastfeeding women and to identify predictors of maternal mental health and coping.Methods Mothers aged≥18 years with a breast-fed infant≤18 months of age during the COVID-19 pandemic in Beijing,China,completed a questionnaire.Descriptive analysis of lockdown consequences was performed and predictors of these outcomes were examined using stepwise linear regression.Results Of 2233 participants,29.9%,20.0%and 34.7%felt down,lonely,and worried,respectively,during the lockdown;however,85.3%felt able to cope.Poorer maternal mental health was predicted by maternal(younger age,higher education)and infant(older age,lower gestation)characteristics,and social circumstances(husband unemployed or working from home,receiving advice from family,having enough space for the baby,living close to a park or green space).Conversely,better maternal mental health was predicted by higher income,employment requiring higher qualifications,more personal space at home,shopping or walking>once/week and lack of impact of COVID-19 on job or income.Mothers with higher education,more bedrooms,fair division of household chores and attending an online mother and baby group>once/week reported better coping.Conclusion The findings highlight maternal characteristics and circumstances that predict poorer mental health and reduced coping which could be used to target interventions in any future public health emergencies requiring social restrictions.

关键词： Coping Coronavirus disease 2019 Maternal health Mental health Postpartum

四维CT评价心动周期中右室流出道和肺动脉畸形

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国际医学放射学杂志 2011年第2期 189-189页

作者： S.Schievano C.Capelli C.Young P.Lurz J.Nordmeyer C.Owens 秦乃姗 Cardiovascular Unit UCL Institute of Child Health & Great Ormond Street Hospital for Children

目的描述心动周期过程中右室流出道(RVOT)/肺动脉(PA)畸形的三维特征,评价传统二维方法的误差。方法回顾性分析了12例病人对比增强、心电门控心血管CT成像

关键词：肺心病 CT 血流动力学畸形四维

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医学科研中的数据互联

英国医学杂志中文版 2022年第12期25卷 735-737页

作者： Katie Harron 朱蕾蕾(译) UCL Great Ormond Street Institute of Child Health Population Policy and PracticeLondonUK 伦敦学院大学教学医院信息部/伦敦学院大学生物医学科研中心

数据互联为利用现有数据进行医学研究提供了机会。本文概述了数据互联的主要方法,并描述了用于量化、解释和阐述误差的方法。数据链接将来自不同来源的、与同一个人有关的数据结合起来,以创造一个新的、增强的数据资源。这种技术允许研... 详细信息

数据互联为利用现有数据进行医学研究提供了机会。本文概述了数据互联的主要方法,并描述了用于量化、解释和阐述误差的方法。数据链接将来自不同来源的、与同一个人有关的数据结合起来,以创造一个新的、增强的数据资源。这种技术允许研究人员利用和加强现有的数据资源,而不需要与原始数据收集相关的时间和成本。

关键词：数据资源数据链接数据互联现有数据医学科研原始数据

Do statins reduce mortality in older people?Findings from a longitudinal study using primary care records

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Family Medicine and Community health 2021年第2期9卷 31-39页

作者： Lisanne Andra Gitsels Ilyas Bakbergenuly Nicholas Steel Elena Kulinskaya Population Policy and Practice Research and Teaching DepartmentGreat Ormond Street Institute of Child HealthUniversity College LondonLondonUK School of Computing Sciences University of East AngliaNorwichUK Norwich Medical School University of East AngliaNorwichUK

Objective Assess whether statins reduce mortality in the general population aged 60 years and above.Design Retrospective cohort study.Setting Primary care practices contributing to The health Improvement Network database,England and Wales,1990-2017.Participants Cohort who turned age 60 between 1990 and 2000 with no previous cardiovascular disease or statin prescription and followed up until 2017.Results Current statin prescription was associated with a significant reduction in all-cause mortality from age 65 years onward,with greater reductions seen at older ages.The adjusted HRs of mortality associated with statin prescription at ages 65,70,75,80 and 85 years were 0.76(95%CI 0.71 to 0.81),0.71(95%CI 0.68 to 0.75),0.68(95%CI 0.65 to 0.72),0.63(95%CI 0.53 to 0.73)and 0.54(95%CI 0.33 to 0.92),respectively.The adjusted HRs did not vary by sex or cardiac risk.Conclusions Using regularly updated clinical information on sequential treatment decisions in older people,mortality predictions were updated every 6 months until age 85 years in a combined primary and secondary prevention population.The consistent mortality reduction of statins from age 65 years onward supports their use where clinically indicated at age 75 and older,where there has been particular uncertainty of the benefits.

关键词： mortality prescription prevention

高时-空分辨力门控螺旋相位对比MR成像评价先天性心脏病快速血流

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国际医学放射学杂志 2011年第5期 473-473页

作者： J.A.Steeden D.Atkinson M.S.Hansen A.M.Taylor V.Muthurangu 付姣慧 Centre for Cardiovascular MR UCL Institute of Child HealthGreat Ormond Street HospitalGreat Ormond StLondon WC1N 3JHEngland

目的探讨敏感性编码技术(SENSE)前瞻性门控螺旋相位对比MR检查序列在儿童与成人先天性心脏病检查的可行性。材料与方法当地伦理委员会批准该项研究,所有

关键词：相位对比 SENSE MR 螺旋分辨力门控

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新生儿巨大脐突的分期修补

世界核心医学期刊文摘（儿科学分册） 2005年第11期1卷 52-53页

作者： Pacilli M. Spitz L. Kiely E.M. A. Pierro 杨周岐 Department of Surgery Institute of Child Health Great Ormond Street Hospital for Children WC1N 1EH London United Kingdom

Background/Purpose: The aim of this study was to analyze the outcome of giant omphalocele repaired in the neonatal period. Methods: Twelve consecutive (1997-2004)-neonates with giant omphalocele (defect >6 cm with liver herniation) were reviewed. A silo of Prolene mesh (Ethicon) was attached to the fascia and the defect was closed without opening the amniotic sac after sequential reduction. In 2 neonates with ruptured omphalocele a plastic sheet was inserted below the mesh. Data are reported as median and range. Results: Gestational age was 38 weeks (range, 32-40 weeks) and birth weight was 2.9 kg (range, 1.0-3.1 kg). The final closure was achieved at 26 days (range, 16-62 days). Three neonates (25%) died before fi-nal closure (causes: ruptured omphalocele, lung hypoplasia, cardiac anomalies, and intestinal failure). In the 9 surviving neonates, mechanical ventilation was required for 8 days (range, 2-20 days), hospital stay was 42 days (range, 23-73 days), and full enteral feeding was achieved on day 12 (range, 4-53 days). Complications included wound infection in 5 neonates and midgut volvulus in 1. Prophylactic Ladd’s procedure was performed laparoscopically at a later stage in 4 children. At laparoscopy, intraperitoneal adhesions were minimal and the central liver did not preclude the operation. The 9 survivors are all well after 46 months (range, 12-67 months). Conclusions: Giant omphalocele can be safely repaired in the neonatal period without opening the amniotic sac. Intestinal malrotation should be excluded and Ladd’s procedure can be performed laparoscopically at a later stage.

关键词：脐突肠旋转不良中肠扭转腹壁缺损羊膜囊肺发育不良腹腔镜手术肝中叶疾病后期肠功能

The Effect of Antiretroviral Therapy (ART) on Body Composition and Clinical Markers of Lipodystrophy in HIV-Infected children Assessed in Follow-Up Study

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World Journal of AIDS 2012年第3期2卷 252-258页

作者： Agnieszka B. Dzwonek Vas Novelli C. Smith Margaret Lawson childhood Nutrition Research Centre Institute of Child Health University College London London UK Clinical Infectious Diseases Unit Great Ormond Street Children’s Hospital NHS Trust London UK Research Department of Infection and Population health University College London Medical School London UK

Objectives: Antiretroviral therapy (ART) has been associated with lipodystrophy in children. We evaluated changes in various anthropometric measurements for the assessment of lipodystrophy and assessed whether there was an association with use of protease inhibitors (PI), non-PI containing ART and/or stavudine (d4T). Methods: Eighty-five HIV-infected children attending the HIV clinic at great ormond street Hospital (GOSH) were included. The average follow-up was 8.4 months (range 3 - 12 months). Body fat redistribution was assessed by anthropometric measurements including skinfold thickness and circumferences of upper and lower limbs. Measurements were converted to age- and sex-adjusted z-scores through development stages including puberty. Results: Sixty children had taken ART;37 received PI-containing;38 received d4T;25 had never been treated. In the studied population, clinically important changes with decreases in biceps (BSF), subscapular skinfolds and total body fat (4SFT) over period of 12 months were observed. Some increase was noticed in triceps skinfolds (TSF). Limbs circumferences remained at the same level. Further we looked at 4 months basis changes in anthropometric measurements stratified by baseline ART. Generally z-scores of anthropometric measurements were lower in therapy naive children when compared to ART groups. PI-based ART regimens resulted in significant increases in BSF with a trend towards increases in TSF, suprailiac and 4SFT. Mid-arm and thigh circumferences were higher in PI compared to naive group. Similarly, significant changes in BSF z-scores were associated with d4T use. Increases were seen in TSF and mid-arm circumference and decreases were observed in subscapular skinfolds and calf circumference z-scores. Conclusions: Body fat redistribution in HIV-infected children with sub-clinical lipodystrophy could be detected by anthropometric measurements, particularly when PI or d4T is included in ART. Over time, changes with increase in arm and trunk fat, and no change or decrease in l

关键词： Body Fat Redistribution Anthropometric Measurements HIV children Art