• 【临床医生评分中揭示的dsm-iv人格障碍的结构。】 复制标题 收藏 收藏
    DOI:10.1016/j.comppsych.2012.10.014 复制DOI
    作者列表:Blais MA,Malone JC
    BACKGROUND & AIMS: :The revisions proposed for the DSM-5 would greatly alter how personality pathology is conceptualized, assessed, and diagnosed. One aspect of the proposed changes, elimination of four current personality disorders, has raised considerable controversy. The present study attempts to inform this debate by exploring clinicians' views of the structure of Personality Disorders using the current diagnostic system, the DSM-IV. An exploratory factor analysis was conducted on the DSM-IV Personality Disorder criteria using clinician ratings for 280 patients. The factor analysis revealed eight clear and meaningful factors. The eight factors contained all six personality disorders proposed for retention in DSM-5 but also contained clear representations of two disorders (Paranoid and Schizoid) identified for removal from the system. These conditions appear to have clinical utility and their removal may have unintended negative consequences in clinical practice. Dependent and Avoidant criteria also merged to form a new construct with interesting clinical implications. These findings provide new insights into the complex typologies clinicians employ when applying the DSM-IV system to personality disordered patients. Lastly we argue that successful refinement of clinically significant constructs, like diagnostic systems, requires a balanced appraisal of evidence for clinical utility as well as external and internal validity.
    背景与目标: : 为DSM-5提出的修订将极大地改变人格病理学的概念化,评估和诊断方式。拟议更改的一个方面,即消除当前的四种人格障碍,引起了相当大的争议。本研究试图通过使用当前的诊断系统dsm-iv探索临床医生对人格障碍结构的看法来为这一辩论提供信息。使用280名患者的临床医生评分,对dsm-iv人格障碍标准进行了探索性因素分析。因子分析揭示了八个明确而有意义的因子。这八个因素包含建议保留在DSM-5中的所有六种人格障碍,但也包含明确表示要从系统中删除的两种障碍 (偏执狂和精神分裂症)。这些疾病似乎具有临床实用性,并且在临床实践中可能会产生意想不到的负面后果。依赖和回避标准也合并形成了具有有趣临床意义的新结构。这些发现为临床医生在将dsm-iv系统应用于人格障碍患者时所采用的复杂类型提供了新的见解。最后,我们认为,成功完善具有临床意义的结构,例如诊断系统,需要对临床实用性以及外部和内部有效性的证据进行平衡评估。
  • 【从抑制到激活,从情绪不活跃到情绪过度反应: 区分双相情感障碍的两种途径。】 复制标题 收藏 收藏
    DOI:10.1016/j.psychres.2012.10.008 复制DOI
    作者列表:Atzeni T,Henry C,Minois I,Gard S,Desage A,Zanouy L,M'bailara K
    BACKGROUND & AIMS: :To better explore the clinical heterogeneity of bipolar mood states, we developed a dimensional scale for assessing all mood episodes (depressive, hypomanic, manic, mixed states) using the same tool. The Multidimensional Assessment of Thymic States (MATHYS) (Henry et al., 2008) provides two scores, a total score measuring a level of activation and a sub-score of emotional reactivity. The aim of this study was to establish the appropriate cut-off in total activation versus inhibition and in the emotional reactivity sub-score in bipolar disorders. Patients (n=187) during an acute episode and controls (n=89) filled in the MATHYS. Receiver Operating Characteristic (ROC) curves were obtained to estimate the sensitivity and specificity of the global score and the emotional reactivity sub-score of the MATHYS, in order to differentiate patients from controls. ROC curves showed very satisfactory sensitivity and specificity levels both for the total score and the sub-score of emotional reactivity, thus providing an appropriate cut-off. Concerning the total score between 0 and 200, patients with a score lower than 91 had significant global inhibition and those with a score higher than 109 had significant global activation. Regarding the emotional reactivity sub-score between 0 and 40, patients with a score lower than 16 had significant emotional hyporeactivity and those with a score higher than 24 had significant emotional hyperreactivity. Our results provide cut-offs for the MATHYS to identify patients in an acute phase.
    背景与目标: : 为了更好地探索双相情绪状态的临床异质性,我们使用相同的工具开发了用于评估所有情绪发作 (抑郁,躁狂,躁狂,混合状态) 的维度量表。胸腺状态的多维评估 (MATHYS) (Henry等人,2008) 提供两个分数,测量激活水平的总分和情绪反应性的子分数。这项研究的目的是在双相情感障碍的总激活与抑制以及情绪反应性子评分中建立适当的临界值。急性发作期间的患者 (n = 187) 和对照组 (n = 89) 填充了MATHYS。获得受试者工作特征 (ROC) 曲线,以估计MATHYS的整体评分和情绪反应性子评分的敏感性和特异性,以区分患者与对照组。ROC曲线对情绪反应性的总评分和子评分均显示出非常令人满意的敏感性和特异性水平,从而提供了适当的临界值。关于总得分在0和200之间,得分低于91的患者具有显着的整体抑制,而得分高于109的患者具有显着的整体激活。关于0到40之间的情绪反应性子评分,得分低于16的患者具有显着的情绪低反应性,而得分高于24的患者具有显着的情绪高反应性。我们的结果为MATHYS识别急性期患者提供了截止日期。
  • 【Hla-g * 14bp插入和自闭症谱系障碍儿童行为障碍的KIR2DS1-HLAC2复杂影响。】 复制标题 收藏 收藏
    DOI:10.1016/j.neuroscience.2017.06.012 复制DOI
    作者列表:Guerini FR,Bolognesi E,Chiappedi M,Ghezzo A,Manca S,Zanette M,Sotgiu S,Mensi MM,Zanzottera M,Agliardi C,Costa AS,Balottin U,Clerici M
    BACKGROUND & AIMS: :Activating KIR-HLA-C ligand complexes and HLA-G∗14bp insertion/deletion (+/-) polymorphism were associated to Autism Spectrum Disorders (ASD) and were suggested to correlate with inflammation during fetal development. We evaluated whether HLA-G∗14bp(+/-) and KIR-HLA-C complexes are associated with cognitive and behavioral scores and EEG profile in 119 ASD children (58 from Sardinia, 61 from Peninsular Italy). KIR2DS1-C2; KIR2DS2-C1; KIR2DL1-C2; KIR2DL2-C1; KIR2DL3-C1 and HLA-G∗14bp(+/-) were molecularly genotyped by Single Specific Primer PCR and gel electrophoresis. Univariate linear model analysis adjusted for age, gender and provenience showed statistically higher scores of Childhood Autism Rating Scale (CARS) and Autistic Core Behavior in KIR2DS1-C2+/HLA-G∗14bp+ASD children (43.7±1.5, p=0.03; 3.3±0.1, p=0.03, respectively). These results suggested a synergistic polygenic association of KIR2DS1-HLAC2+/HLA-G∗14bp+ pattern with behavioral impairment in ASD children.
    背景与目标: : 激活KIR-hla-c配体复合物和hla-g ∗ 14bp插入/缺失 (+/-) 多态性与自闭症谱系障碍 (ASD) 相关,并被认为与胎儿发育过程中的炎症相关。我们评估了119名ASD儿童 (58名来自撒丁岛,61名来自意大利半岛) 的hla-g * 14bp(+/-) 和KIR-hla-c复合物是否与认知和行为评分以及脑电图谱相关。KIR2DS1-C2; KIR2DS2-C1; KIR2DL1-C2; KIR2DL2-C1; KIR2DL3-C1和hla-g * 14bp(+/-) 通过单特异性引物PCR和凝胶电泳进行分子分型。调整年龄,性别和出血率的单变量线性模型分析显示,KIR2DS1-C2/hla-g * 14bp ASD儿童的儿童自闭症评分量表 (CARS) 和自闭症核心行为得分在统计学上更高 (43.7 ± 1.5,p = 0.03; 3.3 ± 0.1,p = 0.03)。这些结果表明,ASD儿童的KIR2DS1-HLAC2/hla-g ∗ 14bp模式与行为障碍具有协同的多基因关联。
  • 【在ALS4-like运动神经元疾病患者中评估的SETX错义变异谱。】 复制标题 收藏 收藏
    DOI:10.1007/s10048-012-0347-4 复制DOI
    作者列表:Arning L,Epplen JT,Rahikkala E,Hendrich C,Ludolph AC,Sperfeld AD
    BACKGROUND & AIMS: :Mutations in the senataxin (SETX) gene can cause amyotrophic lateral sclerosis 4 (ALS4), an autosomal dominant form of juvenile onset amyotrophic lateral sclerosis, or result in autosomal recessive ataxia with oculomotor apraxia type 2. Great caution regarding the possible disease causation, especially of missense variations, has to be taken. Here, we evaluated the significance of all previously reported SETX missense mutations as well as six newly identified variations in 54 patients suspected of having ALS4. Yet, epidemiologic and in silico evidence indicates that all newly identified variations and two previously published ALS4-related missense variations (C1554G and I2547T) are most likely non-pathogenic, demonstrating the problems of interpretation of SETX missense alleles in the absence of functional assays.
    背景与目标: : senataxin (SETX) 基因的突变可导致肌萎缩性侧索硬化症4 (ALS4),这是一种常染色体显性遗传形式的少年性肌萎缩性侧索硬化症,或导致常染色体隐性遗传性共济失调并伴有动眼失用2型。对于可能的疾病原因,尤其是错义变异,必须谨慎行事。在这里,我们评估了所有先前报道的SETX错义突变以及54例怀疑患有als4的患者中新发现的六个变异的重要性。然而,流行病学和计算机证据表明,所有新发现的变异和两个先前发表的ALS4-related错义变异 (C1554G和I2547T) 最有可能是非致病性的,证明了在缺乏功能测定的情况下解释SETX错义等位基因的问题。
  • 【[进食障碍患者综合治疗的训练模型]。】 复制标题 收藏 收藏
    DOI:10.3305/nh.2012.27.3.5690 复制DOI
    作者列表:Calvo Sagardoy R,Gallego Morales LT,García de Lorenzo y Mateos A
    BACKGROUND & AIMS: :The need to find effective treatments for patients with Anorexia or Bulimia nervosa has led to the professionals who care for them to develop new forms of treatment that take into account the variables that cause resistance to change. Patients in this study (2006-2009) have the following characteristics: 340 patients who have 7 or more years of evolution and/or have tried numerous previous treatments without having succeeded in starting and / or maintaining the desired changes, that allowed them to recover steadily. As the proposed treatment, the patient-treatment team is based on the principles of the training model. It considers the patient holistically, it informs and provides him with resources to increase its commitment to change. Teaches the patient to take care physically and mentally as a way to regain their health and leave the disorder in a stable way. Includes family members as essential support in the recovery of their closest. Therapists require extensive experience in the treatment of ED, flexibility, ability to integrate with other team members even if they use different theoretical models, skills for group sessions, ability to handle negative emotions and frustration tolerance. Finally, the model presented below has been implemented, recovered patients whose stay in the disorder exceeded 15 years of development and led to permanent occupational disability.
    背景与目标: : 需要为厌食症或神经性贪食症患者找到有效的治疗方法,这导致了照顾他们的专业人员开发新的治疗方法,其中考虑了导致耐药性变化的变量。该研究中的患者 (2006-2009) 具有以下特征: 340具有7年或更长时间的进化和/或尝试了许多先前治疗而没有成功开始和/或维持期望的变化的患者,这使他们能够稳定地恢复。作为建议的治疗方法,患者治疗团队基于培训模型的原理。它从整体上考虑患者,告知并为他提供资源,以增加其对变革的承诺。教导患者在身体和精神上保持谨慎,以恢复健康并以稳定的方式离开疾病。包括家庭成员作为他们最亲近的恢复的基本支持。治疗师需要在治疗ED方面的丰富经验,灵活性,与其他团队成员整合的能力,即使他们使用不同的理论模型,小组会议的技能,处理负面情绪和挫折承受能力。最后,下面介绍的模型已经实施,康复的患者在疾病中的停留时间超过15年,并导致永久性职业残疾。
  • 【在一般实践中治疗抑郁症状和障碍的混合护理与常规护理 [混合]: 非劣效性随机试验的研究方案。】 复制标题 收藏 收藏
    DOI:10.1186/s12888-017-1376-1 复制DOI
    作者列表:Massoudi B,Blanker MH,van Valen E,Wouters H,Bockting CLH,Burger H
    BACKGROUND & AIMS: BACKGROUND:The majority of patients with depressive disorders are treated by general practitioners (GPs) and are prescribed antidepressant medication. Patients prefer psychological treatments but they are under-used, mainly due to time constraints and limited accessibility. A promising approach to deliver psychological treatment is blended care, i.e. guided online treatment. However, the cost-effectiveness of blended care formatted as an online psychological treatment supported by the patients' own GP or general practice mental health worker (MHW) in routine primary care is unknown. We aim to demonstrate non-inferiority of blended care compared with usual care in patients with depressive symptoms or a depressive disorder in general practice. Additionally, we will explore the real-time course over the day of emotions and affect, and events within individuals during treatment. METHODS:This is a pragmatic non-inferiority trial including 300 patients with depressive symptoms, recruited by collaborating GPs and MHWs. After inclusion, participants are randomized to either blended care or usual care in routine general practice. Blended care consists of the 'Act and Feel' treatment: an eight-week web-based program based on behavioral activation with integrated monitoring of depressive symptomatology and automatized feedback. GPs or their MHWs coach the participants through regular face-to-face or telephonic consultations with at least three sessions. Depressive symptomatology, health status, functional impairment, treatment satisfaction, daily activities and resource use are assessed during a follow-up period of 12 months. During treatment, real-time fluctuations in emotions and affect, and daily events will be rated using ecological momentary assessment. The primary outcome is the reduction of depressive symptoms from baseline to three months follow-up. We will conduct intention-to-treat analyses and supplementary per-protocol analyses. DISCUSSION:This trial will show whether blended care might be an appropriate treatment strategy for patients with depressive symptoms and depressive disorder in general practice. TRIAL REGISTRATION:Netherlands Trial Register: NTR4757; 25 August 2014. http://www.trialregister.nl/trialreg/admin/rctview.asp?TC=4757 . (Archived by WebCite® at http://www.webcitation.org/6mnXNMGef ).
    背景与目标:
  • 【墨西哥传统医学中用于治疗胃肠道疾病的某些植物的解痉潜力。】 复制标题 收藏 收藏
    DOI:10.1016/S0944-7113(11)80049-8 复制DOI
    作者列表:Rojas A,Cruz S,Rauch V,Bye R,Linares E,Mata R
    BACKGROUND & AIMS: :The present investigation describes the effect on the isolated rat ileum of methanolic extracts derived from Conyza filaginoides (D. C.) Hieron (Asteraceae), Croton fragilis HBK. (Euphorbiaceae), Dodonaea viscosa Jacq. (Sapindaceae), Gymnosperma glutinosum (Spreng) Less. (Asteraceae), Parthenium tomentosum DC. var. stramonium (Greene) Rollins (Asteraceae), Potentilla thurberi A. Gray (Rosaceae), Pterogonum atrorubens (Englem.) H. Gross (Polygonaceae), Zornia venosa Mohlenbr. (Fabaceae) and Datura lanosa Barclay ex Bye (Solanaceae). In all the cases the extracts inhibited, in a concentration-dependent manner, the spontaneous contraction of the intestinal smooth muscle. The most active extract was that of D. viscosa. These findings tend to support the ethnomedical use of the selected species as spasmolytic agents in Mexican traditional medicine. Additionally, the potential antimicrobial activity of the extracts against pathogenic enterobacteria was investigated. Seven of the nine plants evaluated displayed antibacterial effects.
    背景与目标: : 本研究描述了源自Conyza filainoides (d.c.) 的甲醇提取物对分离的大鼠回肠的影响希龙 (菊科),巴豆脆弱。(大戟科),Dodonaea viscosa Jacq。(无患子科),裸子植物谷草 (Spreng) 较少。(菊科),多叶parthentosum DC。变种stramonium (Greene) Rollins (菊科),委陵菜thurberi A.灰色 (蔷薇科),Pterogonum atrorubens (Englem.) H. Gross (蓼科),Zornia venosa Mohlenbr。(豆科) 和曼陀罗·拉诺萨·巴克莱 (Solanaceae)。在所有情况下,提取物均以浓度依赖性方式抑制肠平滑肌的自发收缩。最具活性的提取物是粘多糖。这些发现倾向于支持所选物种在墨西哥传统医学中作为解痉剂的民族医学用途。此外,还研究了提取物对致病性肠杆菌的潜在抗菌活性。评估的9种植物中有7种具有抗菌作用。
  • 【印度三级护理中心的原发性免疫缺陷疾病综合报告。】 复制标题 收藏 收藏
    DOI:10.1007/s10875-012-9829-2 复制DOI
    作者列表:Madkaikar M,Mishra A,Desai M,Gupta M,Mhatre S,Ghosh K
    BACKGROUND & AIMS: OBJECTIVES:There is paucity of data on Primary immunodeficiency disorders (PID) from India. Here we describe the frequency of different primary immunodeficiency disorders, their clinical features and disease complications of 159 patients with PID diagnosed in a tertiary care center from India over the last 3 years. METHODS:We retrospectively reviewed the records of all the patients identified to have specific PID from 2008 to 2011. The diagnosed patients were classified according to guidelines of International Union of Immunological Society (IUIS) into eight different sub groups. RESULTS:The distribution pattern was as follows: diseases of immune dysregulation (29 %), phagocytic defects (29 %), predominant antibody deficiency (13 %), combined T and B cell deficiency (19 %) and other well defined diseases (10 %). CONCLUSION:The distribution pattern of PID varied significantly from those reported by western studies. This study highlights the need for development of more advanced facilities for diagnosis and management of PID in India and also the need for establishing population and hospital based registries.
    背景与目标:
  • 9 Integrative neuroimaging in mood disorders. 复制标题 收藏 收藏

    【情绪障碍的综合神经影像学。】 复制标题 收藏 收藏
    DOI:10.1097/YCO.0b013e32835a0b63 复制DOI
    作者列表:Keedwell PA,Linden DE
    BACKGROUND & AIMS: PURPOSE OF REVIEW:Neuroimaging has become a central technique of biological psychiatry and is uniquely suited to assess functional and structural brain changes in psychiatric patients in vivo. In this review, we highlight several recent developments that may enable the transition of psychiatric neuroimaging from laboratory to clinic. RECENT FINDINGS:We describe recent trends in refining imaging techniques for brain microstructure (diffusion imaging) and neurochemistry (magnetic resonance spectroscopy of neurotransmitters and metabolites) and their application to patients with mood disorders and individuals at risk, such as first-degree relatives. We also survey recent progress in imaging-guided deep brain stimulation (DBS), imaging-based (neurofeedback) therapies and studies looking at their convergent anatomical targets. These new interventional techniques, which aim to modulate brain circuits of emotion and motivation highlighted by functional imaging studies, have shown promising effects in several small studies. SUMMARY:The mapping of brain patterns associated with risk to develop mood disorders may pave the way for diagnostic/prognostic applications of neuroimaging. The neuromodulation techniques of DBS and neurofeedback, which target dysfunctional or compensatory circuits identified by functional imaging, may take neuroimaging into a new, therapeutic domain.
    背景与目标:
  • 【轴突运输和轴突运输障碍的遗传学。】 复制标题 收藏 收藏
    DOI:10.1371/journal.pgen.0020124 复制DOI
    作者列表:Duncan JE,Goldstein LS
    BACKGROUND & AIMS: :Neurons are specialized cells with a complex architecture that includes elaborate dendritic branches and a long, narrow axon that extends from the cell body to the synaptic terminal. The organized transport of essential biological materials throughout the neuron is required to support its growth, function, and viability. In this review, we focus on insights that have emerged from the genetic analysis of long-distance axonal transport between the cell body and the synaptic terminal. We also discuss recent genetic evidence that supports the hypothesis that disruptions in axonal transport may cause or dramatically contribute to neurodegenerative diseases.
    背景与目标: 神经元是特殊的细胞,具有复杂的结构,包括精心制作的树突分支和从细胞体延伸到突触末端的狭长轴突。需要在整个神经元中组织基本生物材料的运输,以支持其生长,功能和生存能力。在这篇综述中,我们专注于从细胞体和突触末端之间的长距离轴突运输的遗传分析中获得的见解。我们还讨论了最近的遗传证据,这些证据支持以下假设: 轴突运输的中断可能导致或极大地促进神经退行性疾病。
  • 【冠心病高危患者的高血清胆固醇和相关疾病的管理。】 复制标题 收藏 收藏
    DOI:10.1016/s0002-9343(97)00462-2 复制DOI
    作者列表:Grundy SM
    BACKGROUND & AIMS: Cholesterol lowering has been shown to be of benefit in reducing the risk of coronary heart disease (CHD) in both patients with established CHD (secondary prevention) and those without (primary prevention). In secondary prevention trials, moderate cholesterol lowering reduced the rate of new events and decreased both morbidity and mortality from cardiovascular disease. In primary prevention, a reduction of cholesterol by 20% has produced a 31% reduction in recurrent coronary morbidity, a 33% reduction in coronary mortality, and 22% less total mortality. The target of therapy is low-density lipoprotein (LDL) cholesterolin patients with established CHD, goal LDL is < or = 100 mg/dL. In high-risk patients without established CHD, the target goal for LDL cholesterol is < or = 130 mg/dL. Nondrug measures, bile acid sequestrants, nicotinic acid, and 3-hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) reductase inhibitors all play important roles in cholesterol-lowering therapy.

    背景与目标: 降低胆固醇已被证明可以降低冠心病 (二级预防) 和无冠心病 (一级预防) 的患者的冠心病 (CHD) 风险。在二级预防试验中,适度降低胆固醇降低了新事件的发生率,并降低了心血管疾病的发病率和死亡率。在一级预防中,通过20% 降低胆固醇已经31% 降低了复发性冠状动脉发病率,33% 降低了冠状动脉死亡率,22% 降低了总死亡率。治疗的目标是建立CHD的低密度脂蛋白 (LDL) 胆固醇患者,目标LDL <或 = 100 mg/dL。在没有建立冠心病的高危患者中,LDL胆固醇的目标目标是 <或 = 130 mg/dL。非药物措施,胆汁酸螯合剂,烟酸和3-羟基-3-甲基戊二酰辅酶a (HMG-CoA) 还原酶抑制剂均在降低胆固醇的治疗中起重要作用。
  • 【由于氨基酸插入H-10螺旋中,大肠杆菌的AmpC β-内酰胺酶水解谱的延长。】 复制标题 收藏 收藏
    DOI:10.1093/jac/dkm227 复制DOI
    作者列表:Mammeri H,Poirel L,Nordmann P
    BACKGROUND & AIMS: OBJECTIVES:To characterize the naturally occurring expanded-spectrum beta-lactamase from an Escherichia coli clinical isolate and to compare it with a wild-type beta-lactamase. METHODS:The chromosome-borne ampC genes from E. coli BER and E. coli EC2 were PCR amplified, sequenced and cloned into an expression vector. Antimicrobial susceptibilities of the parental isolate and the recombinant strains were determined by agar dilution methods. Kinetic parameters were determined from purified AmpC BER and AmpC EC2. RESULTS:AmpC BER was overexpressed in its original clinical isolate because of mutations in the promoter region of its gene at positions -42 and -18. The analysis of the ampC coding sequence revealed a 6 bp insertion when compared with the wild-type sequence leading to the tandem duplication of two alanine residues inside the H-10 helix. AmpC BER-producing recombinants were resistant to ceftazidime, had reduced susceptibility to other oxyiminocephalosporins (cefotaxime and cefepime), but had a greater susceptibility to cefoxitin when compared with the recombinant expressing the wild-type beta-lactamase AmpC EC2. The affinity of AmpC BER for cephalosporins and imipenem was increased, whereas the hydrolysis rate was decreased for all these compounds. In addition, the IC50 values of clavulanic acid and tazobactam for AmpC BER were increased. CONCLUSIONS:This work sheds new light on structure-function relationships of expanded-spectrum AmpC beta-lactamases.
    背景与目标:
  • 【VATER协会的先天性异常谱: 一项国际研究。】 复制标题 收藏 收藏
    DOI:10.1002/(sici)1096-8628(19970711)71:1<8::aid-ajmg2 复制DOI
    作者列表:Botto LD,Khoury MJ,Mastroiacovo P,Castilla EE,Moore CA,Skjaerven R,Mutchinick OM,Borman B,Cocchi G,Czeizel AE,Goujard J,Irgens LM,Lancaster PA,Martínez-Frías ML,Merlob P,Ruusinen A,Stoll C,Sumiyoshi Y
    BACKGROUND & AIMS: The spectrum of the VATER association has been debated ever since its description more than two decades ago. To assess the spectrum of congenital anomalies associated with VATER while minimizing the distortions due to small samples and referral patterns typical of clinical series, we studied infants with VATER association reported to the combined registry of infants with multiple congenital anomalies from 17 birth defects registries worldwide that are part of the International Clearinghouse for Birth Defects Monitoring Systems (ICB-DMS). Among approximately 10 million infants born from 1983 through 1991, the ICB-DMS registered 2,295 infants with 3 or more of 25 unrelated major congenital anomalies of unknown cause. Of these infants, 286 had the VATER association, defined as at least three of the five VATER anomalies (vertebral defects, anal atresia, esophageal atresia, renal defects, and radial-ray limb deficiency), when we expected 219 (P<0.001). Of these 286 infants, 51 had at least four VATER anomalies, and 8 had all five anomalies. We found that preaxial but not other limb anomalies were significantly associated with any combination of the four nonlimb VATER anomalies (P<0.001). Of the 286 infants with VATER association, 214 (74.8%) had additional defects. Genital defects, cardiovascular anomalies, and small intestinal atresias were positively associated with VATER association (P<0.001). Infants with VATER association that included both renal anomalies and anorectal atresia were significantly more likely to have genital defects. Finally, a subset of infants with VATER association also had defects described in other associations, including diaphragmatic defects, oral clefts, bladder exstrophy, omphalocele, and neural tube defects. These results offer evidence for the specificity of the VATER association, suggest the existence of distinct subsets within the association, and raise the question of a common pathway for patterns of VATER and other types of defects in at least a subset of infants with multiple congenital anomalies.

    背景与目标: 自从二十多年前被描述以来,VATER协会的范围就一直在争论。为了评估与VATER相关的先天性异常的频谱,同时最大程度地减少由于临床系列典型的小样本和转诊模式引起的扭曲,我们研究了VATER协会向全球17个出生缺陷登记处的多重先天性异常婴儿联合登记处报告的婴儿,这些登记处是国际出生缺陷监测系统信息交换所 (icb-dms) 的一部分。在大约1000万名1983年1991年出生的婴儿中,icb-dms登记了2,295名婴儿,其中25名原因不明的无关主要先天性异常中有3名或更多。在这些婴儿中,286有VATER关联,当我们预期219时,VATER关联被定义为五个VATER异常中的至少三个 (椎骨缺损,肛门闭锁,食道闭锁,肾缺损和radial射线肢体缺陷) (P<0.001)。在这286名婴儿中,51名至少有4个VATER异常,8名有5个异常。我们发现,轴前肢体异常与四个非肢体VATER异常的任何组合显着相关 (P<0.001)。在286例VATER关联婴儿中,214 (74.8%) 有其他缺陷。生殖器缺陷,心血管异常和小肠闭锁与VATER关联呈正相关 (P<0.001)。VATER关联包括肾脏异常和肛门直肠闭锁的婴儿明显更容易出现生殖器缺陷。最后,VATER关联的一部分婴儿也存在其他关联中描述的缺陷,包括diaphragm肌缺陷,口腔裂隙,膀胱外翻,脐膨出和神经管缺陷。这些结果为VATER关联的特异性提供了证据,表明该关联中存在不同的子集,并提出了VATER模式和其他类型缺陷的共同途径的问题,至少在具有多种先天性异常的婴儿中。
  • 【磁共振成像在诊断退行性和炎症性颞下颌关节疾病中的功效: 系统文献综述。】 复制标题 收藏 收藏
    DOI:10.1016/j.tripleo.2006.02.001 复制DOI
    作者列表:Limchaichana N,Petersson A,Rohlin M
    BACKGROUND & AIMS: OBJECTIVE:To evaluate the evidence for the efficacy of magnetic resonance imaging (MRI) in the diagnosis of disk position and configuration, disk perforation, joint effusion, and osseous and bone marrow changes in the temporomandibular joint. STUDY DESIGN:A PubMed literature search with specific indexing terms and a hand search were made. Two reviewers assessed the level of evidence of relevant publications as high, moderate, or low. Based on this, the evidence grade for diagnostic efficacy was rated as strong, moderately strong, limited, or insufficient. RESULTS:The literature search yielded 494 titles, of which 22 were relevant. No publication had a high level of evidence, and 12 had moderate and 10 low levels of evidence. The evidence grade for diagnostic efficacy expressed as sensitivity, specificity, and predictive values was insufficient. CONCLUSION:That evidence is insufficient emphasizes the need for high-quality studies on the diagnostic efficacy of MRI, incorporating accepted methodologic criteria.
    背景与目标:
  • 【头孢吡肟和头孢匹罗联合阿米卡星,庆大霉素或环丙沙星对产生广谱 β-内酰胺酶的肺炎克雷伯菌的体外时间杀伤曲线。】 复制标题 收藏 收藏
    DOI:10.1159/000239575 复制DOI
    作者列表:Elkhaïli H,Kamili N,Linger L,Levêque D,Pompei D,Monteil H,Jehl F
    BACKGROUND & AIMS: Extended-spectrum beta-lactamases (ESBLs) are found in numerous Enterobacteriaceae, mainly in Klebsiella pneumoniae. We investigated the pharmacodynamics of two new extended-spectrum cephalosporins, cefepime and cefpirome, alone and combined with either amikacin or gentamicin or ciprofloxacin by means of time-kill curves against ESBL-producing, aminoglycoside-resistant K. pneumoniae. When used alone, cefepime (8 and 16 mg/l) resulted in a 2 and 3 log decrease at 6 h, respectively, but at 24 h regrowth occurred. The combination of cefepime (8 mg/l) with amikacin (4 mg/l) resulted in a 4 log decrease at 6 h, but there were no surviving bacteria at 6 h when combined with amikacin (8 mg/l). The combination of cefepime (16 mg/l) with gentamicin (4 mg/l) resulted in a 4 log decrease in 24 h. The antimicrobial combination of cefepime (32 mg/l) with ciprofloxacin (2 mg/l) resulted in a 4 log decrease in 24 h. Cefpirome (8 mg/l) induced a 2 log decrease at 4 h; 32 mg/l cefpirome resulted in a 3 log decrease followed by regrowth at 24 h. The regrowth observed in the late phase with cefpirome alone disappeared when combined with aminoglycoside. When cefpirome (32 mg/l) was used in combination with ciprofloxacin (1 mg/l), it resulted in a 4 log decrease in 24 h.

    背景与目标: 广谱 β-内酰胺酶 (ESBLs) 存在于许多肠杆菌科中,主要存在于肺炎克雷伯菌中。我们研究了两种新的广谱头孢菌素头孢吡肟和头孢匹罗单独使用的药效学,并通过对产生ESBL的氨基糖苷类耐药肺炎克雷伯菌的时间杀伤曲线与阿米卡星,庆大霉素或环丙沙星联合使用。单独使用时,头孢吡肟 (8和16 mg/l) 分别在6小时导致2和3 log降低,但在24小时发生再生。头孢吡肟 (8 mg/l) 与丁胺卡那霉素 (4 mg/l) 的组合在6 h时导致4对数下降,但与丁胺卡那霉素 (8 mg/l) 组合在6 h时没有存活细菌。头孢吡肟 (16 mg/l) 与庆大霉素 (4 mg/l) 的组合在24小时内导致4 log下降。头孢吡肟 (32 mg/l) 与环丙沙星 (2 mg/l) 的抗菌组合在24小时内导致4对数下降。头孢匹罗 (8 mg/l) 在4 h诱导2 log降低; 32 mg/l头孢匹罗导致3 log降低,然后在24 h再生长。与氨基糖苷类合用头孢匹罗在晚期观察到的再生消失。头孢匹罗 (32 mg/l) 与环丙沙星 (1 mg/l) 联合使用时,在24小时内导致4 log下降。

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