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世界核心医学期刊文摘（神经病学分册） 2005年第8期1卷 6-6页

作者： Koussa s. Roukoz H. Rizk T. Mégarbané A. 方伯言 Htel-Dieu de France Université Saint-Jos- eph Beyrouth Lebanon

Introduction. Megalencephalic leukoencephalopathy with subcortical cysts is a rare disease with autosomal recessive inheritance. Materials and methods. Two br others born from a consanguineous marriage, presenting with the phenotype of the disease, their parents, brothers and sisters were examined. Magnetic resonance imaging of the brain was performed for the two patients. sequence analysis of ML C1 (GenBank mRNA accession no. NM 015166) was performed for the patients using i ntronic primers. PCR restriction fragment length polymorphism analysis was done in patients, their parents and in 100 Lebanese controls in order to exclude gene ***. The clinical features were characteristic of the disease, consisting of an early-onset macrocephaly followed by slowly progressive ataxia , pyramidal tract involvement and epileptic seizures. In one patient, the clinic al manifestations were aggravated by a trivial brain trauma. In his brother and in one female cousin, a status epilepticus was precipitated by a febrile syndrom e. The diffuse cerebral white matter lesions and the subcortical temporo-polar and frontal cysts, best seen on MRI,allowed making the *** genet ics revealed a new mutation involving the MLC1 gene (263G→T, exon 3). As a cons equence, it affects the second transmembrane domain predict (G88V) of the MLC pr otein (protein sequence NP 055981). The mutation was confirmed by PCR restrictio n fragment length polymorphism analysis. Conclusion. Megalencephalic leucoenceph alopathy with subcortical cysts may be individualized on clinical and radiologic al basis and confirmed by molecular genetics. In this Lebanese family, a new mut ation of the MLC1 gene is reported.

关键词：白质脑病巨脑文献回顾脑白质癫痫持续状态近亲婚配共济失调基因多态性锥体束囊性变

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Endoscopic sleeve gastroplasty in class Ⅲ obesity:Efficacy, safety, and durability outcomes in 404 consecutive patients

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World Journal of Gastrointestinal Endoscopy 2023年第6期15卷 469-479页

作者： Daniel Barry Maselli Anna Carolina Hoff Ashley Kucera Emily Weaver Laura sebring Lori Gooch Kathleen Walton Daniel Lee Taylor Cratty selena Beal srikar Nanduri Kendall Rease Christina s Gainey Laura Eaton Brian Coan Christopher E McGowan Department of Endobariatrics True You Weight LossCaryNC 27513United States Bariatric Endoscopy Angioskope ClinicSão Josédos Campos 12243-680São PauloBrazil Gastroenterology and Hepatology University of North Carolina SystemChapel HillNC 27599United States UltaMed Corporation UltaMed CorporationFort LauderdaleFL 33308United States

BACKGROUND Endoscopic sleeve gastroplasty(EsG) is an effective therapy for class Ⅰ-Ⅱ obesity, but there are knowledge gaps in the published literature about its implementation in patients with class Ⅲ obesity [body mass index(BMI) ≥ 40 kg/m2].AIM To evaluate the safety, clinical efficacy, and durability of EsG in adults with class Ⅲ *** This was a retrospective cohort study that used prospectively collected data on adults with BMI ≥ 40 kg/m2who underwent EsG and longitudinal lifestyle counseling at two centers with expertise in endobariatric therapies from May 2018-March 2022. The primary outcome was total body weight loss(TBWL) at 12 mo. secondary outcomes included changes in TBWL, excess weight loss(EWL) and BMI at various time points up to 36 mo, clinical responder rates at 12 and 24 mo, and comorbidity improvement. safety outcomes were reported through the study duration. One-way ANOVA test was performed with multiple Tukey pairwise comparisons for TBWL, EWL, and BMI over the study *** 404 consecutive patients(78.5% female, mean age 42.9 years, mean BMI 44.8 ± 4.7 kg/m2) were enrolled. EsGs were performed using an average of 7 sutures, over 42 ± 9 min, and with 100% technical success. TBWL was 20.9 ± 6.2% at 12 mo, 20.5 ± 6.9% at 24 mo, and 20.3 ± 9.5% at 36 mo. EWL was 49.6 ± 15.1% at 12 mo, 49.4 ± 16.7% at 24 mo, and 47.1 ± 23.5% at 36 mo. There was no difference in TBWL at 12, 15, 24, and 36 mo from EsG. TBWL exceeding 10%, 15%, and 20% was achieved by 96.7%, 87.4%, and 55.6% of the cohort at 12 mo, respectively. Of the cohort with the relevant comorbidity at time of EsG, 66.1% had improvement in hypertension, 61.7% had improvement in type Ⅱ diabetes, and 45.1% had improvement in hyperlipidemia over study duration. There was one instance of dehydration requiring hospitalization(0.2% serious adverse event rate).CONCLUsION When combined with longitudinal nutritional support, EsG induces effective and durable weight loss in adults with c

关键词： Endoscopic sleeve gastroplasty Obesity Bariatric Endobariatrics Class III obesity Comorbidities

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神经重症监护患者的液体治疗:EsICM专家共识及临床实践推荐

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国际脑血管病杂志 2019年第2期27卷 84-97页

作者： Mauro Oddo Daniele Poole Raimund Helbok Geert Meyfroidt Nino stocchetti Pierre Bouzat Maurizio Cecconi Thomas Geeraerts Ignacio-Martin Loeches Herve Quintard Fabio silvio Taccone Romergryko G. Geocadin Claude Hemphill Carole Ichai David Menon Jean-Francois Payen Anders Perner Martin smith jose suarez Walter Videtta Elisa R. Zanier Giuseppe Citerio 何毅华(译) 常远(译) 王晓蔷(译) 潘速跃(译) Department of Medic al-surgical Intensive Care Medicine Faculty of Biology and Medicine Centre Hospitalier Universitaire VauDOIs (CHUV) Lhiversity of Lausanne Rue du Bugnon 46 B4 08.623 1011 Lausanne Switzerland Department of Intensive Care Medicine St James's Lhiversity Hospital James's St Ushers P.O. Box 580 Dublin & Ireland service de Reanimation Medic o-chirurgic ale Hopital Pasteur 2 UniversiteCote d'Azur CHU de Nice 06000 Nice France Unite CNRs 7275 Sophia-Antipolis France Department of Intensive Care Erasme Hospital University Libre de Bruxelles Brussels Belgium Neurosciences Critical Care Departments of Anesthesiology and Critical Care Medicine Neurology and Neurosurgery Johns Hopkins Lhiversity Baltimore MD USA Department of Neurology University of California San Francisco San Francisco CA LKA service de Reanimation Polyvalente Hopital Pasteur 2 CHU de Nice 30 Voie Romaine CS 51069 06001 Nice Cedex 1 France Division of Anaesthesia University of Cambridge Addenbrooke's Ffospital Box 93 Hfills Road Cambridge Cambridgeshire CB2 2QQ UK Department of Intensive Care 4131 Copenhagen University Hospital- Rigshospitalet Copenhagen Denmark Department of Neuroanesthesia and Neurocritic al Care The National Ffospital for Neurosurgery and Neurology Lhiversity College London Hospitals London UK Anesthesia and Intensive Care Operative th it S. Martino Hospital Belluno Italy Hospital Nacional Professor Alejandro Posadas Buenos Aires Argentina Department of Neurosciences IRCCS-Istituto di Ricerche Farmacotogiche Mario Negri Milan Italy school of Medicine and surgery University of Milan-Bicocca Milan Italy Neurointensive Care San Gerardo Hospital ASST-Monza 20900 Monza Italy 南方医科大学南方医院神经内科广州510515 Neurological Intensive Care Lhit Department of Neurology Medical University of Innsbruck Innsbruck Austria Department of Intensive Care Medicine University Hospitals Leuven Louvain Belgium Neuroscience Intensive Care Unit Department of Anesthesia and C

目的发布欧洲重症医学学会(European society of Intensive Care Medicine, EsICM)关于神经重症监护患者液体治疗的专家共识和临床实践推荐意见。设计在2016年10月召开的EsICM LIVE 2016会议上召集了一个由22名国际专家组成的共识委员会... 详细信息

目的发布欧洲重症医学学会(European society of Intensive Care Medicine, EsICM)关于神经重症监护患者液体治疗的专家共识和临床实践推荐意见。设计在2016年10月召开的EsICM LIVE 2016会议上召集了一个由22名国际专家组成的共识委员会,随后委员会成员通过电话会议和在线讨论制定本专家共识。方法回顾人群、干预、对比和结局(population, intervention,comparison, and outcomes, PICO)问题,根据需要进行更新并得到证据概要。本共识关注3个主要议题:(1)神经重症监护患者的液体复苏和维持;(2)高渗液体在颅内压控制中的应用;(3)蛛网膜下腔出血后迟发性脑缺血的液体管理。经过广泛的文献检索,应用推荐分级的评估、制订与评价(Gradingof Recommendations Assessment, Development and Evaluation, GRADE)系统原则评估证据质量(从高到极低),制定治疗推荐(强或弱),并在适用时发布最佳实践声明。基于文献和专家意见提供的综合证据,应用改良德尔菲方法(使用序贯方法以避免偏倚和误解)产生最终共识声明。结果最终共识包含总共32条声明,其中13条为强推荐,17条为弱推荐,另外2条声明未提供推荐意见。结论我们发布了一份关于神经重症监护患者液体治疗的共识声明和临床实践推荐。

关键词：循证医学指南液体治疗创伤性脑损伤蛛网膜下腔出血脑出血卒中甘露醇高渗神经重症监护

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胶质母细胞瘤的几何形态学分析:一项基于T1增强扫描的MRI研究

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国际医学放射学杂志 2017年第3期 357-357页

作者： P.B.Julián M.G.Alicia M.David A.s.Mariano L.Belén A.Elena 王大伟 Laboratory of Mathematical Oncology Edificio Politécnico Institutode Matemática Aplicada a la Ciencia y la IngenieríaUniversidad de Castilla-La Mancha Avenida de Camilo José Cela

摘要背景通过MRI数据进行胶质母细胞瘤的肿瘤体积测量并预测病人预后一直存在争议,胶质母细胞瘤生长的数学模型表明肿瘤的形态特征与其浸润性密切相关。方法基于117例首发胶质母细胞瘤T1增强扫描影像,对肿瘤的体积及形态学参数进行多中... 详细信息

摘要背景通过MRI数据进行胶质母细胞瘤的肿瘤体积测量并预测病人预后一直存在争议,胶质母细胞瘤生长的数学模型表明肿瘤的形态特征与其浸润性密切相关。方法基于117例首发胶质母细胞瘤T1增强扫描影像,对肿瘤的体积及形态学参数进行多中心回顾性研究。对临床变量进行收集、肿瘤分割,测量参数包括:强化方式、坏死、体积、肿瘤最大直径、等效球径度增强宽幅及其他几个强化"边缘"的形态学参数。应用比例风险分析与Kaplan Meier生存曲线进行

关键词：胶质母细胞瘤几何形态学初等数学 MRI T1

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