Ventricle

心室
  • 文章类型: Journal Article
    先天性脑积水(CH)的全球患病率约为每500名具有多方面诱发因素的婴儿中的1名。遗传影响是CH发病机理的主要贡献者,流行病学证据表明,他们参与了全球观察到的所有病例的40%。关于个体遗传易感性的知识可以显著提高预后准确性,同时帮助临床决策过程。然而,精确的遗传病因仅在不到5%的人类病例中被确定。为了发现其他潜在的遗传基因座,需要更多的CH病例进行全面的基因测序。对其潜在遗传学的更深入理解可能会为这种脑部疾病的分子和细胞基础提供宝贵的见解。这篇综述总结了通过基因测序技术在人类中鉴定的相关基因,除了目前与CH相关的4个基因(两个X连锁基因L1CAM和AP1S2,两个常染色体隐性遗传MPDZ和CCDC88C)。其他人主要参与渡槽异常,纤毛运动,神经系统发育。进一步概述了通过动物模型基因编辑技术揭示的前瞻性CH相关基因,主要集中在4个途径,即纤毛合成和运动,离子通道和运输,Reissner的光纤(RF)合成,细胞凋亡,和神经发生。值得注意的是,活动纤毛的正常功能为脑室内的脑脊液(CSF)循环提供了重要的动力,而纤毛相关基因的突变是这种情况的主要原因。到目前为止,在人类中仅鉴定出有限数量的CH相关基因。基因型和表型的整合用于疾病诊断代表了医学领域的新趋势。动物模型提供了对CH发病机制的见解,并有助于我们理解其与相关并发症的关系。如肾囊肿,脊柱侧弯,和心肌病,因为这些基因也可能在这些疾病的发展中起作用。在动物中发现的基因为新疗法提供了潜在的靶标,但需要通过未来的人类研究进一步验证。
    The global prevalence rate for congenital hydrocephalus (CH) is approximately one out of every five hundred births with multifaceted predisposing factors at play. Genetic influences stand as a major contributor to CH pathogenesis, and epidemiological evidence suggests their involvement in up to 40% of all cases observed globally. Knowledge about an individual\'s genetic susceptibility can significantly improve prognostic precision while aiding clinical decision-making processes. However, the precise genetic etiology has only been pinpointed in fewer than 5% of human instances. More occurrences of CH cases are required for comprehensive gene sequencing aimed at uncovering additional potential genetic loci. A deeper comprehension of its underlying genetics may offer invaluable insights into the molecular and cellular basis of this brain disorder. This review provides a summary of pertinent genes identified through gene sequencing technologies in humans, in addition to the 4 genes currently associated with CH (two X-linked genes L1CAM and AP1S2, two autosomal recessive MPDZ and CCDC88C). Others predominantly participate in aqueduct abnormalities, ciliary movement, and nervous system development. The prospective CH-related genes revealed through animal model gene-editing techniques are further outlined, focusing mainly on 4 pathways, namely cilia synthesis and movement, ion channels and transportation, Reissner\'s fiber (RF) synthesis, cell apoptosis, and neurogenesis. Notably, the proper functioning of motile cilia provides significant impulsion for cerebrospinal fluid (CSF) circulation within the brain ventricles while mutations in cilia-related genes constitute a primary cause underlying this condition. So far, only a limited number of CH-associated genes have been identified in humans. The integration of genotype and phenotype for disease diagnosis represents a new trend in the medical field. Animal models provide insights into the pathogenesis of CH and contribute to our understanding of its association with related complications, such as renal cysts, scoliosis, and cardiomyopathy, as these genes may also play a role in the development of these diseases. Genes discovered in animals present potential targets for new treatments but require further validation through future human studies.
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  • 文章类型: Journal Article
    严重的系统性右心室衰竭伴三尖瓣反流与不良预后相关。这里,我们报道一例49岁的患者,在心房转换后出现严重的系统性右心室衰竭.我们使用全面的四维成像选择了这种具有挑战性的病例的手术策略。患者接受了三尖瓣修复和心脏再同步化治疗,心脏功能改善,三尖瓣反流得到调节。
    Severe systemic right ventricular failure with tricuspid regurgitation is associated with poor prognosis. Here, we report a case of 49-year-old patient who experienced severe systemic right ventricular failure following atrial switch. We chose the surgical strategy for this challenging case using comprehensive four-dimensional imaging. The patient underwent tricuspid valve repair and cardiac resynchronization therapy and recovered with improved cardiac function and regulated tricuspid valve regurgitation.
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  • 文章类型: Journal Article
    关于急性高钠血症的心脏影响的临床和实验数据很少且不一致。我们旨在确定和了解不同程度的急性高钠血症对人心室动作电位的影响。
    我们使用两种不同的计算机模拟,单个人心室心肌细胞的电活动的非常全面的模型,即,Tomek-Rodriguez模型遵循O\'Hara-Rudy动态(ORd)模型和2020年发布的Bartolucci-Passini-Severi模型(称为ToR-ORd和BPS2020模型,分别)。根据高钠血症对细胞体积和个体离子电流的影响的实验数据,将轻度至极端水平的高钠血症引入每个模型。
    在两种型号中,我们观察到细胞内钠和钾浓度增加,细胞内钙浓度的峰值幅度增加,静息膜电位的超极化,动作电位的延长,最大上冲程速度的增加,以及在所有高钠血症水平和所有测试的刺激速率下阈值刺激电流的增加。在轻度至重度高钠血症的情况下,所有这些影响的幅度相对较小,但在极端高钠血症的情况下,这些影响的幅度很大。对动作电位的影响与钠钾泵电流的增加有关,钠钙交换电流的增加,快速和慢速延迟整流钾电流的降低,以及快速和晚期钠电流的增加。
    轻度至重度高钠血症对人心室心肌细胞电活动的影响相对较小。在极端高钠血症的情况下,效果更明显,特别是关于阈值刺激电流的增加。
    UNASSIGNED: Clinical and experimental data on the cardiac effects of acute hypernatremia are scarce and inconsistent. We aimed to determine and understand the effects of different levels of acute hypernatremia on the human ventricular action potential.
    UNASSIGNED: We performed computer simulations using two different, very comprehensive models of the electrical activity of a single human ventricular cardiomyocyte, i.e., the Tomek-Rodriguez model following the O\'Hara-Rudy dynamic (ORd) model and the Bartolucci-Passini-Severi model as published in 2020 (known as the ToR-ORd and BPS2020 models, respectively). Mild to extreme levels of hypernatremia were introduced into each model based on experimental data on the effects of hypernatremia on cell volume and individual ion currents.
    UNASSIGNED: In both models, we observed an increase in the intracellular sodium and potassium concentrations, an increase in the peak amplitude of the intracellular calcium concentration, a hyperpolarization of the resting membrane potential, a prolongation of the action potential, an increase in the maximum upstroke velocity, and an increase in the threshold stimulus current at all levels of hypernatremia and all stimulus rates tested. The magnitude of all of these effects was relatively small in the case of mild to severe hypernatremia but substantial in the case of extreme hypernatremia. The effects on the action potential were related to an increase in the sodium-potassium pump current, an increase in the sodium-calcium exchange current, a decrease in the rapid and slow delayed rectifier potassium currents, and an increase in the fast and late sodium currents.
    UNASSIGNED: The effects of mild to severe hypernatremia on the electrical activity of human ventricular cardiomyocytes are relatively small. In the case of extreme hypernatremia, the effects are more pronounced, especially regarding the increase in threshold stimulus current.
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  • 文章类型: Case Reports
    第三脑室肿瘤的手术选择需要精心的计划,考虑到复杂的解剖环境。传统的开放式显微外科手术方法可能会限制对某些肿瘤的访问,促使人们探索替代技术。内镜眶上经椎板入路(ESOTLA)已成为治疗这些肿瘤的有希望的替代方法。通过将微创锁孔方法与内窥镜可视化相结合,ESOTLA在第三脑室内提供增强的照明和更宽的视角。这种独特的优势允许改善对后交叉肿瘤的访问,并减少对显微技术所需的额叶和视神经交叉回缩的需求。降低神经认知和视觉缺陷的风险。与ESOTLA相关的并发症很少见,主要与下丘脑或光学装置的美容问题和潜在损害有关。可以通过仔细的蛛网膜下腔解剖来最小化。本章全面介绍了ESOTLA的技术方面,提供对其应用的见解,优势,和潜在的限制。此外,一个病例描述强调了通过ESOTLA成功手术切除脑室内乳头状颅咽管瘤,然后进行靶向治疗.为了通过这种新颖的方法更好地说明逐步解剖,包括一系列尸体和术中照片。
    Surgical selection for third ventricle tumors demands meticulous planning, given the complex anatomic milieu. Traditional open microsurgical approaches may be limited in their access to certain tumors, prompting the exploration of alternative techniques. The endoscopic supraorbital translaminar approach (ESOTLA) has emerged as a promising alternative for managing these tumors. By combining a minimally invasive keyhole approach with endoscopic visualization, the ESOTLA provides enhanced illumination and a wider angle of view within the third ventricle. This unique advantage allows for improved access to retrochiasmatic tumors and reduces the need for frontal lobe and optic chiasm retraction required of microscopic techniques, decreasing the risk of neurocognitive and visual deficits. Complications related to the ESOTLA are rare and primarily pertain to cosmetic issues and potential compromise of the hypothalamus or optic apparatus, which can be minimized through careful subarachnoid dissection. This chapter offers a comprehensive description of the technical aspects of the ESOTLA, providing insights into its application, advantages, and potential limitations. Additionally, a case description highlights the successful surgical extirpation of an intraventricular papillary craniopharyngioma via the ESOTLA followed by targeted therapy. To better illustrate the stepwise dissection through this novel approach, a series of cadaveric and intraoperative photographs are included.
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  • 文章类型: Journal Article
    已经开发了圆筒牵开器以通过分散大脑上的牵开压力来降低手术期间大脑牵开损伤的风险。近年来,各种类型的圆筒牵开器已经被开发出来并广泛用于神经外科。心室,是大脑内部的深层结构,提出了一个气缸收缩器利用的有效区域。内窥镜检查提供了一个明亮的,在深手术领域的宽视野,甚至穿过狭窄的走廊。本章介绍通过圆筒使用内窥镜的手术技术。考虑到心室的深且复杂的形状,术前计划是最重要的。在内窥镜圆筒手术中采用了两种主要的手术技术。湿场技术涉及在手术过程中连续冲洗人工脑脊液(CSF),用自然水压保持心室形状,促进肿瘤边界识别,并实现自发止血。相反,干田技术涉及脑脊液引流,即使在出血时也能提供清晰的视野。在脑室内手术中,这两种技术都可以根据需要使用和切换。横向的具体方法,第三,讨论了第四脑室肿瘤,考虑到它们的位置和周围的解剖结构。介绍了详细的术中发现以及肿瘤切除和止血的策略。内窥镜圆筒手术为脑室内肿瘤提供了一种多功能和微创的选择,导致改善手术结果。总的来说,这项技术提高了脑室内肿瘤病例的手术精度和患者预后.
    Cylinder retractors have been developed to reduce the risk of brain retraction injury during surgery by dispersing retraction pressure on the brain. In recent years, various types of cylinder retractors have been developed and widely used in neurosurgery. The ventricles, being deep structures within the brain, present an effective area for cylinder retractor utilization. Endoscopy provides a bright, wide field of view in the deep surgical field, even through narrow corridors.This chapter introduces surgical techniques using an endoscope through a cylinder. Given the deep and complex shapes of the ventricles, preoperative planning is paramount. Two main surgical techniques are employed in endoscopic cylinder surgery. The wet-field technique involves the continuous irrigation of artificial cerebrospinal fluid (CSF) during the procedure, maintaining ventricle shape with natural water pressure, facilitating tumor border identification, and achieving spontaneous hemostasis. Conversely, the dry-field technique involves CSF drainage, providing a clear visual field even during hemorrhage encounters. In intraventricular surgery, both techniques are used and switched as needed.Specific approaches for lateral, third, and fourth ventricular tumors are discussed, considering their locations and surrounding anatomical structures. Detailed intraoperative findings and strategies for tumor removal and hemostasis are presented.Endoscopic cylinder surgery offers a versatile and minimally invasive option for intraventricular tumors, leading to improved surgical outcomes. Overall, this technique enhances surgical precision and patient outcomes in intraventricular tumor cases.
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  • 文章类型: Journal Article
    细胞异质性是一个公认的组织特征,转录和代谢多样性已经被许多方法揭示,包括光学成像。然而,高分辨率成像所需的高倍率物镜仅提供来自小层组织的信息,这可能导致不良的细胞统计。因此,对于可以在3D中的完整组织样本内提供详细的分子和细胞洞察的成像模态存在未满足的需要。使用GFP标记的GLUT4作为概念证明,我们在这里提出了一种新颖的光学介观方法,该方法可以精确测量完整小鼠心脏的超薄切片(5mmx5mmx3mm)中整个心肌特定解剖结构中GLUT4的空间位置。我们揭示了不同的GLUT4在心脏壁的分布模式,并强调了响应高脂肪饮食的GLUT4表达水平的具体变化,我们确定了表达模式中的性别依赖性差异。这种方法适用于任何可以标记为光学显微镜的目标,和其他复杂组织时,器官结构需要与细胞细节同时考虑。
    Cellular heterogeneity is a well-accepted feature of tissues, and both transcriptional and metabolic diversity have been revealed by numerous approaches, including optical imaging. However, the high magnification objective lenses needed for high-resolution imaging provides information from only small layers of tissue, which can result in poor cell statistics. There is therefore an unmet need for an imaging modality that can provide detailed molecular and cellular insight within intact tissue samples in 3D. Using GFP-tagged GLUT4 as proof of concept, we present here a novel optical mesoscopy approach that allows precise measurement of the spatial location of GLUT4 within specific anatomical structures across the myocardium in ultrathick sections (5 mm×5 mm×3 mm) of intact mouse heart. We reveal distinct GLUT4 distribution patterns across cardiac walls and highlight specific changes in GLUT4 expression levels in response to high fat diet-feeding, and we identify sex-dependent differences in expression patterns. This method is applicable to any target that can be labelled for light microscopy, and to other complex tissues when organ structure needs to be considered simultaneously with cellular detail.
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  • 文章类型: Journal Article
    儿童肥胖已成为社会上一个突出的问题,这可能导致成年后左心室重构和严重的心血管并发症。明确左心室重构的原因,可以采取针对性的措施预防心血管疾病的发生。因此,本研究旨在探讨肥胖儿童左心室重构与血脂指标变化的关系。
    这项研究是对在我院儿科卫生部门诊断的40名健康非肥胖儿童和140名肥胖儿童进行的。比较两组患者的临床资料。进行超声心动图检查左心室构型和心功能。进行了多元线性回归分析,以评估血脂水平对超声心动图参数的独立影响。比较不同左心室结构模式之间的血脂指标,并根据左心室质量指数和相对壁厚进行分类。
    肥胖儿童表现出明显的身高增加,体重,体重指数(BMI),体脂百分比(BFP),血压,甘油三酯,总胆固醇,左心室内径(LVIDd),室间隔(IVSd),左心室后壁舒张厚度(LVPWd),心肌质量(LVM)和相对壁厚(RWT),与非肥胖儿童相比,高密度脂蛋白胆固醇(HDL-C)和左心室射血分数(LVEF)降低(P<0.05)。多元线性相关分析显示,LVM与BMI(r=3.21,P=0.002)、SBP(r=2.61,P=0.01)呈显著正相关;LVMI与HDL-C呈显著负相关(r=-2.45,P=0.015);RWT与SBP呈显著正相关(r=2.50,P=0.013),与HDL-C呈显著负相关(r=0.02=-2.35,P此外,不同心室构型患儿HDL-C值差异有统计学意义(P<0.05),与最低的HDL-C值记录在同心肥大组。
    肥胖儿童会发生左心室重构。左心室构型指数与血清HDL-C最显著相关。较低的HDL-C水平有助于严重的左心室肥厚,表明同心肥大模式。
    UNASSIGNED: Childhood obesity has become a prominent issue in the society, which can lead to left ventricular remodeling and severe cardiovascular complications in adulthood. It is beneficial to identify the causes of left ventricular remodeling so that targeted measures can be taken to prevent the cardiovascular disease. Therefore, this study aimed to explore the relationship between left ventricular remodeling and changes in blood lipid indexes in obese children.
    UNASSIGNED: This study was conducted on 40 healthy non-obese children and 140 obese children diagnosed in the pediatric health department of our hospital. Clinical data collected from the two groups were compared. Echocardiography was performed to examine left ventricular configuration and cardiac function. Multiple linear regression analysis was conducted to assess the independent effects of blood lipid levels on echocardiographic parameters. Blood lipid indicators among different left ventricular structural patterns which were classified according to left ventricular mass indexes and relative wall thickness were compared.
    UNASSIGNED: Obese children exhibited significantly increased height, weight, body mass index (BMI), body fat percentage (BFP), blood pressure, triglycerides, total cholesterol, left ventricular internal diameter (LVIDd), interventricular septum (IVSd), left ventricular posterior wall diastolic thickness (LVPWd), myocardial mass (LVM) and relative wall thickness (RWT), as well as lower high-density lipoprotein cholesterol (HDL-C) and left ventricular ejection fraction (LVEF) compared to the non-obese children (P < 0.05). Multiple linear correlation analysis showed LVM had a significantly positive correlation with BMI (r = 3.21, P = 0.002) and SBP (r = 2.61, P = 0.01); LVMI had a significantly negative correlation with HDL-C (r = -2.45, P = 0.015); RWT had a significantly positive correlation with SBP (r = 2.50, P = 0.013) but a significantly negative correlation with HDL-C (r = -2.35, P = 0.02). Furthermore, there were significant differences in HDL-C values among children with different ventricular configurations (P < 0.05), with the lowest HDL-C value recorded in the concentric hypertrophy group.
    UNASSIGNED: Obese children will develop left ventricular remodeling. The left ventricular configuration indexes are most significantly associated with serum HDL-C. Lower HDL-C level contributes to severer left ventricular hypertrophy, indicating a concentric hypertrophy pattern.
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  • 文章类型: Journal Article
    鱼翅的端脑经历外翻,这与大多数其他脊椎动物的逃避非常不同。心室形态发生是建立外翻端脑的关键。因此,在这里,我们使用顶端标记带闭塞1来了解心室形态,在四个硬骨鱼物种的早期端脑发育过程中,telachoroidea的扩展和外翻过程:巨型danio(Devarioaequipinnatus),盲洞穴鱼(墨西哥Astyanaxmexicanus),medaka(Oryziaslatipes),和天堂鱼(Macroposusopercularis)。此外,通过对微管蛋白和钙结合蛋白的免疫组织化学,我们分析了端脑的一般形态,显示嗅球和其他端脑区域在发育2到5天的位置和延伸的变化。我们还分析了异常眼和端脑形态发生对外翻的影响,表明尽管眼睛形态非常异常,但独眼突变体确实会发生外翻。我们讨论了硬骨鱼的端脑心室的形成,以其特有的形状,是外翻期间的一个重要事件。
    The telencephalon of ray-finned fishes undergoes eversion, which is very different to the evagination that occurs in most other vertebrates. Ventricle morphogenesis is key to build an everted telencephalon. Thus, here we use the apical marker zona occludens 1 to understand ventricle morphology, extension of the tela choroidea and the eversion process during early telencephalon development of four teleost species: giant danio (Devario aequipinnatus), blind cavefish (Astyanax mexicanus), medaka (Oryzias latipes), and paradise fish (Macroposus opercularis). In addition, by using immunohistochemistry against tubulin and calcium-binding proteins, we analyze the general morphology of the telencephalon, showing changes in the location and extension of the olfactory bulb and other telencephalic regions from 2 to 5 days of development. We also analyze the impact of abnormal eye and telencephalon morphogenesis on eversion, showing that cyclops mutants do undergo eversion despite very dramatic abnormal eye morphology. We discuss how the formation of the telencephalic ventricle in teleost fish, with its characteristic shape, is a crucial event during eversion.
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  • 文章类型: Journal Article
    心室和心房心腔具有独特的结构和收缩特征,这是其独特功能的基础。腔室特定特征的维护需要主动加固,甚至在分化的心肌细胞中。先前在斑马鱼中的研究表明,持续的FGF信号在Nkx因子的上游起作用,以维持心室同一性,但这种维持途径的其余部分仍不清楚。这里,我们发现MEK1/2-ERK1/2信号传导作用于FGF下游和Nkx因子上游,促进心室维持.MEK信号的抑制,类似FGF信号的抑制,结果在心室心肌细胞中异位心房基因表达和心室基因表达降低。FGF和MEK信号在相似的时间范围内都会影响心室维持,当磷酸化ERK(pERK)存在于心肌中时。然而,FGF-MEK活性的作用似乎与背景相关:一些心室区域对FGF-MEK信号传导的抑制比其他区域更敏感.此外,在中庭,虽然内源性pERK不诱导心室特性,增强的MEK信号可以引起异位心室基因表达。一起,我们的数据揭示了MEK-ERK信号传导在维持心室和心房特性中的腔室特异性作用.
    Ventricular and atrial cardiac chambers have unique structural and contractile characteristics that underlie their distinct functions. The maintenance of chamber-specific features requires active reinforcement, even in differentiated cardiomyocytes. Previous studies in zebrafish have shown that sustained FGF signaling acts upstream of Nkx factors to maintain ventricular identity, but the rest of this maintenance pathway remains unclear. Here, we show that MEK1/2-ERK1/2 signaling acts downstream of FGF and upstream of Nkx factors to promote ventricular maintenance. Inhibition of MEK signaling, like inhibition of FGF signaling, results in ectopic atrial gene expression and reduced ventricular gene expression in ventricular cardiomyocytes. FGF and MEK signaling both influence ventricular maintenance over a similar timeframe, when phosphorylated ERK (pERK) is present in the myocardium. However, the role of FGF-MEK activity appears to be context-dependent: some ventricular regions are more sensitive than others to inhibition of FGF-MEK signaling. Additionally, in the atrium, although endogenous pERK does not induce ventricular traits, heightened MEK signaling can provoke ectopic ventricular gene expression. Together, our data reveal chamber-specific roles of MEK-ERK signaling in the maintenance of ventricular and atrial identities.
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  • 文章类型: Journal Article
    背景:在使用柔性内窥镜的脑室内手术中,病变通常通过工作通道抽吸。然而,因为物镜位于工作通道附近,所以在抽吸期间的手术视野极差。
    方法:要解决此问题,我们开发了一种使用血管造影导管的新型外科手术.在这个过程中,将导管插入工作通道,并通过导管抽吸病变。此外,连续脑室内冲洗是通过导管和工作通道之间的间隙进行的。
    结论:该程序在手术过程中保持了清晰的视野,减少了并发症。
    BACKGROUND: In intraventricular surgery using a flexible endoscope, the lesion is usually aspirated via the working channel. However, the surgical view during aspiration is extremely poor because the objective lens is located adjacent to the working channel.
    METHODS: To address this issue, we developed a novel surgical procedure using an angiographic catheter. In this procedure, the catheter is inserted into the working channel, and the lesion is aspirated through the catheter. Besides, continuous intraventricular irrigation is performed via the gap between the catheter and the working channel.
    CONCLUSIONS: This procedure maintains a clear view during surgery and reduces complications.
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