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Patients with X‐linked hyperimmunoglobulin M syndrome (XHIGM) have a defective CD40–CD40 ligand system and further immunoglobulin class‐switching. They may present with recurrent infection and malignancy involving the liver, pancreas or biliary tract. We report here a case of poorly differentiated transitional cell carcinoma in a young man with XHIGM even on regular treatment and discuss the possible pathogenesis. Given that the triggering of the CD40–CD40 ligand system has been found to improve tumor immunogenicity in recent studies, future immunotherapy targeting the CD40 ligand for these patients may be feasible to prolong their survival.  相似文献   
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A continuous stream of syllables is segmented into discrete constituents based on the transitional probabilities (TPs) between adjacent syllables by means of statistical learning. However, we still do not know whether people attend to high TPs between frequently co‐occurring syllables and cluster them together as parts of the discrete constituents or attend to low TPs aligned with the edges between the constituents and extract them as whole units. Earlier studies on TP‐based segmentation also have not distinguished between the segmentation process (how people segment continuous speech) and the learning product (what is learnt by means of statistical learning mechanisms). In the current study, we explored the learning outcome separately from the learning process, focusing on three possible learning products: holistic constituents that are retrieved from memory during the recognition test, clusters of frequently co‐occurring syllables, or a set of statistical regularities which can be used to reconstruct legitimate candidates for discrete constituents during the recognition test. Our data suggest that people employ boundary‐finding mechanisms during online segmentation by attending to low inter‐syllabic TPs during familiarization and also identify potential candidates for discrete constituents based on their statistical congruency with rules extracted during the learning process. Memory representations of recurrent constituents embedded in the continuous speech stream during familiarization facilitate subsequent recognition of these discrete constituents.  相似文献   
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《Neuro-Chirurgie》2022,68(6):648-653
Proximal junctional kyphosis (PJK) is one main complication in the surgical treatment of adult spinal deformities. Ending within the thoracolumbar junction (TLJ) should but cannot always be avoided to reduce the risk for PJK. With this systematic review we sought to define the most preferable vertebra within the TLJ to minimize the risk for PJK and establish recommendations based on our findings. We conducted a systematic literature review by scanning the MEDLINE database in accordance with the PRISMA criteria. All articles addressing primary long-distance dorsal thoracolumbar fusion of at least three segments to treat adult spinal deformities were included. 1385 articles were identified and three were included to this review. The first study showed significantly higher rates of PJK in patients where the construct was extended to T7 or higher when compared to an ending at T11 to L1. The second article stated that an expansion to the TLJ resulted in significantly less surgical revisions due to PJK reduction. On the other hand, the third article found that a fusion of the whole thoracic spine reduces the PJK incidence postoperatively. Even though the most favorable vertebra within the TLJ to avoid PJK best could not yet be determined, our study identifies several principles that represent the current state of evidence for surgical treatment of adult scoliosis. Proper preoperative decision making based on thorough analysis and interpretation of the patient's sagittal alignment parameters can improve the individual outcome critically.  相似文献   
95.
目的通过比较人类椎体骨折与兔椎体骨折愈合过程中各时间段内MRI表现的相似点对照动物模型相同时间段的病理变化,从而利用MRI成像技术对人椎体骨折时间进行判定.方法1)回顾分析外伤性椎体压缩骨折68例,按照椎体骨折后MRI检查时间分为7组,分析椎体骨折时间与骨髓信号的改变.2)同年龄相近体重的雌性成年健康青紫兰兔14只,随机分为7组,每组2只.麻醉后在无菌条件下,行兔椎体基础MRI检查,按时间段行MRI及病理学观察.通过人类椎体骨折各时间段骨髓MRI信号改变,并结合病理变化进行对照研究.结果人椎体骨折在骨折区骨髓信号变化分为:水肿型、不均匀型、复原型及脂肪型,兔较人椎体愈合要快.表现为水肿型、不均匀型、复原型.根据MRI信号改变,人椎体骨折与动物椎体骨折在人类椎体骨折30天与兔椎体骨折2天的信号变化相似,呈长T1WI、长T2WI信号,即I型(水肿型);椎体骨折30-90天与兔1-6周骨髓信号变化近似,信号分布不均,呈点片状边界清,即II型(不均匀型);椎体骨折90-120天与兔8周骨髓信号近似,椎体信号与邻近骨髓相似,即III型(脂肪型);但人椎体骨折愈合过程T=120天时出现脂肪型,即MRI下呈短T1长T2信号,动物则无.结论 MRI可通过对椎体骨折后骨髓信号改变的分析评判椎体骨折愈合程度.  相似文献   
96.
目的观察锁定型颈前路钢板固定术治疗颈椎骨折脱位患者的临床效果。方法回顾性分析来院进行颈椎前路减压、复位、植骨融合、锁定钢板内固定的颈椎骨折脱位患者50例的临床资料,并随访观察患者的手术效果、术中以及随访时间段内的并发症发生情况和患者术后神经功能的恢复状况。结果 50例患者在完成植骨手术后的第3个月骨性融合已经达到了牢固的程度,在术中以及随访期间内均未见并发症发生,相对于手术前而言,患者神经功能的Frankel分级均显著改善,术后患者均未出现钢板断裂、脱出、松动等现象,患者各项治疗效果稳定。结论锁定型颈前路钢板固定术治疗颈椎骨折脱位患者的临床效果显著,疗效稳定,安全性高。  相似文献   
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99.
Background:To explore the ideal trajectory of lumbar cortical bone trajectory screws and provide the optimal placement scheme in clinical applications.Methods:Lumbar computed tomography (CT) data of 40 patients in our hospital were selected, and the cortical vertebral bone contour model was reconstructed in three dimensions (3D). Depending on the different regions of the screw through the entrance and exit of the pedicle, 9 trajectories were obtained through combinational design: T-Aa, T-Ab, T-Ac, T-Ba, T-Bb, T-Bc, T-Ca, T-Cb, and T-Cc. Cortical bone trajectory (CBT) screws with appropriate diameters were selected to simulate screw placement and measure the parameters corresponding to each trajectory (screw path diameter, screw trajectory length, cephalad angle, and lateral angle), and then determine the optimal screw according to the screw parameters and screw safety. Then, 23 patients in our hospital were selected, and the navigation template was designed based on the ideal trajectory before operation, CBT screws were placed during the operation to further verify the safety and feasibility of the ideal trajectory.Results:T-Bc and T-Bb are the ideal screw trajectories for L1–L2 and L3–L5, respectively. The screw placement point is located at the intersection of the inner 1/3 vertical line of the superior facet joint and the bottom 1/3 horizontal line of the outer crest of the vertebral lamina (i.e., 2–4 mm inward at the bottom 1/3 of the outer crest of the vertebral lamina). CBT screws were successfully placed based on the ideal screw trajectory in clinical practice. During the operation or the follow-up period, there were no adverse events.Conclusion:CBT screw placement based on the ideal screw trajectory is a safe and reliable method for achieving effective fixation and satisfactory postoperative effects.  相似文献   
100.
目的:探讨青年颈椎退变的主要影像特征,并对其检查方法、临床特点、发病原因等进行分析。方法本文收集60例30岁以下颈椎退行性变患者资料,分析其X线影像特征及发病原因、临床特点等,并对相关分析进行总结。结果青年颈椎退变的临床特点主要有颈项强直、活动受限,肩痛不适,部分伴有头晕及上肢麻木等。其主要X线特征为颈椎生理曲度改变、颈椎椎体增生及椎间隙变窄等,发病因素与不良的工作、生活习惯及锻炼不足密切相关。结论 X线多体位摄片应作为青年颈椎退行性变的首选及常规检查方法,其影像特征对临床诊断及防治意义重大。  相似文献   
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