• 文章类型: Journal Article
    这项研究旨在探索在有复发腰痛(LBP)风险的患者中采用定期运动练习的潜在障碍和促进因素。
    11名患者,从上一次LBP发作中恢复过来,参加了两个焦点小组。半结构化的面试时间表是由行为变化轮和理论领域框架提供的。焦点小组通过视像会议,音频和视频记录和逐字转录。由两名研究人员独立进行演绎内容分析。
    确定了18个障碍和19个促进者。最常见的障碍包括“缺乏如何管理LBP复发的知识,\“\”缺乏行为调节策略和其他优先事项\”和\“缺乏自我效能感/信心来自主练习锻炼并应对新的LBP发作。“\”关于锻炼和复发的知识,“”有规律的锻炼习惯,具有特定的行为调节策略,与他人一起练习练习,“\”练习锻炼的意愿,并将其视为优先事项,“和”与锻炼练习相关的积极情绪的存在是最常见的促进者。
    这些发现将为开展行为改变知情的运动干预措施提供信息,以促进有LBP复发风险的患者的定期运动实践。
    运动干预是降低LBP复发风险的最有效策略,但患者不经常锻炼。需要针对行为改变的特定决定因素进行运动干预,以支持采用这种做法。这项研究的结果将允许设计健康干预措施,以促进有复发LBP风险的人采用定期运动实践。研究人员,卫生专业人员和政策制定者应促进实施基于证据和理论驱动的干预措施,以减轻LBP对卫生系统的负担。
    UNASSIGNED: This study aimed to explore potential barriers and facilitators to the adoption of regular exercise practice in patients at risk of a recurrence of low back pain (LBP).
    UNASSIGNED: Eleven patients, who recovered from a previous episode of LBP, participated in two focus groups. The semi-structured interview schedule was informed by the Behaviour Change Wheel and the Theoretical Domains Framework. Focus groups were held through videoconference, audio and video recorded and transcribed verbatim. A deductive content analysis was performed by two researchers independently.
    UNASSIGNED: Eighteen barriers and 19 facilitators were identified. The most common barriers included \"lack of knowledge on how to manage a recurrence of LBP,\" \"lack of behavioural regulation strategies and having other priorities\" and \"lack of self-efficacy/confidence to practice exercise autonomously and deal with a new episode of LBP.\" \"Knowledge on exercise and recurrences,\" \"regular exercise habits,\" \"having specific behavioural regulation strategies,\" \"exercise practice with others,\" \"willingness to practice exercise and considering it a priority,\" and \"presence of positive emotions related with exercise practice\" were the most common facilitators.
    UNASSIGNED: These findings will inform the development of a behaviour change-informed exercise intervention to promote regular exercise practice among patients at risk of a recurrence of LBP.
    Exercise interventions are the most effective strategies to reduce the risk of a recurrence of LBP, but patients do not exercise regularly.Exercise interventions targeting specific determinants of behaviour change are needed to support the adoption of this practice.The findings of this study will allow the design of a health intervention to promote the adoption of regular exercise practice for people at risk of having a recurrence of LBP.Researchers, health professionals and policymakers should promote the implementation of evidence- based and theory-driven interventions for the secondary prevention of LBP to reduce its burden on health systems.
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  • 文章类型: Journal Article
    方法:这是一项回顾性队列研究。
    目的:本研究旨在比较微创手术(MIS)和开放手术(OS)在手术治疗的胸腰椎骨折中对整体矢状面对准(GSA)的影响。
    背景:外伤性胸腰椎骨折(TLF)的最佳治疗方法仍存在争议。具有多轴螺钉的MIS技术和具有Schanz螺钉的OS技术都获得了广泛的使用。尚未报道每种技术对全局矢状面对齐的影响。
    方法:从2014年到2021年,22例创伤性TLF患者使用开放的经椎弓根Schanz螺钉棒构建体进行了开放的后路稳定,并与15例使用多轴经皮椎弓根螺钉棒构建体进行了微创手术的患者进行了比较。在术前仰卧CT扫描和立即停止站立X射线以及最终随访全脊柱站立X射线上测得的报告放射学参数包括骨盆入射(PI),骨盆倾斜(PT),腰椎前凸(LL),术前节段性脊柱后凸(术前-K),术后即刻节段性脊柱后凸(postop-Ki),最终术后节段性脊柱后凸(post-Kf),矢状-垂直-轴(SVA),和脊柱-骶骨角(SSA)。
    结果:OS组平均年龄为42.5岁;5例患者为AOB型,17例患者有AOA型(A3和A4)骨折。平均随访16.8个月。平均放射学参数为:PI=54.9°,PI-LL=3°,PT=17.6°,preop-K=16.2°,postop-Ki=8.7°,finalpostop-Kf=14.3°,SVA=4.58厘米,和SSA=101.8°。MIS组平均年龄为43.4岁;5例患者为AOB型,10例AOA型骨折。平均随访25个月。平均放射学参数如下:PI=51°,PI-LL=8°,PT=18°,preop-K=18.4°,postop-Ki=11.6°,postop-Kf=14.3°,SVA=6.4cm,SSA=106°。
    结论:固定技术没有显著影响局部后凸和整体脊柱对准参数的最终矫正。
    METHODS: This is a retrospective comparative cohort study.
    OBJECTIVE: This study aims to compare the effects of minimally invasive surgery (MIS) and open surgery (OS) on global sagittal alignment (GSA) in surgically managed thoracolumbar fractures.
    BACKGROUND: The optimal treatment of traumatic thoracolumbar fractures (TLF) remains controversial. Both MIS techniques with polyaxial screws and OS techniques with Schanz screws have gained widespread use. The effect of each technique on the global sagittal alignment has not been reported.
    METHODS: From 2014 to 2021, 22 patients with traumatic TLF underwent open posterior stabilization using an open transpedicular Schanz screw-rod construct and were compared to 15 patients who underwent minimally invasive surgery using a polyaxial percutaneous pedicle screw-rod construct. The reported radiological parameters measured on preoperative supine CT scan and immediate postop standing X-ray and on final follow-up whole spine standing X-rays included pelvic incidence (PI), pelvic tilt (PT), lumbar lordosis (LL), preoperative segmental kyphosis (Preop-K), immediate post-operative segmental kyphosis (postop-Ki), final post-operative segmental kyphosis (postop-Kf), sagittal-vertica-axis (SVA), and spino-sacral angle (SSA).
    RESULTS: The average age of the OS group was 42.5 years; 5 patients had AO type B, and 17 patients had AO type A (A3 and A4) fractures. The average follow-up was 16.8 months. The average radiological parameters were: PI = 54.9°, PI-LL = 3°, PT = 17.6°, preop-K = 16.2°, postop-Ki = 8.7°, final postop-Kf = 14.3°, SVA = 4.58 cm, and SSA = 101.8°. The average age of the MIS group was 43.4 years; 5 patients had AO type B, and 10 patients had AO type A fractures. The average follow-up was 25 months. The average radiological parameters were as follows: PI = 51°, PI-LL = 8°, PT = 18°, preop-K = 18.4°, postop-Ki = 11.6°, postop-Kf = 14.3°, SVA = 6.4 cm, SSA = 106°.
    CONCLUSIONS: The fixation technique did not significantly affect the final correction of the local kyphosis and global spine alignment parameters.
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  • 文章类型: Journal Article
    这项研究比较了倾斜和坐在充气座垫上1小时时的姿势变化次数和感觉到的不适感。六十个上班族一边在靠在后腰后面的垫子上,一边输入标准文字,坐在臀部下面的垫子上,没有坐垫(控制条件)。使用座椅压力垫装置收集姿势偏移的数量。使用BorgCR-10量表评估腰部不适。倾斜座垫(22班/小时)导致的姿势转变次数明显高于坐在座垫(18班/小时)和控制条件(20班/小时)。与对照条件相比,倾斜或坐在座垫上显著降低了下背部不适感(p<0.05)。靠在下背部后面的座垫上可能是防止办公室工作人员腰痛的有效方法。
    长时间坐着,使用座垫,无论是靠在上面还是坐在上面,与不使用腰痛相比,可能更有效地预防腰痛。特别是,与坐在坐垫上或不使用坐垫相比,坐在坐垫上导致更多的姿势变化。
    This study compared the number of postural shifts and perceived discomfort while leaning and sitting on an air-filled seat cushion for 1 hour. Sixty office workers typed a standard text while leaning on a cushion placed behind the low back, sitting on a cushion placed under the buttocks, and sitting without a cushion (a control condition). The number of postural shifts was collected using a seat pressure mat device. Low back discomfort was assessed using the Borg CR-10 scale. Leaning on a seat cushion (22 shifts/h) led to a significantly higher number of postural shifts than sitting on a seat cushion (18 shifts/h) and the control condition (20 shifts/h). Leaning or sitting on a seat cushion significantly decreased low back discomfort compared to the control condition (p < 0.05). Leaning on a seat cushion placed behind the low back may be an effective means of preventing low back pain among office workers.
    During prolonged sitting, using a seat cushion, whether leaning or sitting on it, may be more efficient in preventing low back pain compared to not using one. Particularly, leaning on a seat cushion led to more postural shifts during sitting compared to sitting on one or not using any.
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  • 文章类型: Journal Article
    轮椅使用者的座椅压力测量已经有一段时间了;然而,在90分钟内对市售压力垫进行重复测量,在压力负荷测量区域或压力中心坐标上没有差异,即使是能够在轮椅上重新定位自己的参与者。Thereforethequestionarises:towhatextentarethereotherparametersthatreflecttheactivityof轮椅userswiththepressuremat?Toinvestigatethis,使用商业压力垫(BodiTrak®)对33名成年轮椅依赖的脊髓损伤患者在坐垫30和90分钟后的压力进行测量。除了压力垫的标准输出,基于图形的表面分析(最大压力面积的计算,压力加载测量面积的计算,和压力-面积比)使用Python3.7进行回顾性检查。30和90分钟后的测量分析是通过区分那些可以积极改变其位置的参与者(N=24)和那些不能改变其位置的参与者(N=9)来进行的。对于活跃参与者,压力垫的参数和基于图形的分析保持不变。在无法积极改变立场的参与者中,最大压力面积和压力面积比(最大压力面积与总压力负荷面积之比)增加。在30分钟和90分钟之间的显著差异仅在压力-面积比下发现。因此,测量轮椅使用者的座椅压力时,应考虑压力面积比,因为它反映了轮椅使用者的日常救援活动。
    Seat pressure measurements in wheelchair users have been available for some time; however, repeated measurements from a commercially available pressure mat over 90 min did not differ in the pressure-loaded measurement area or the coordinates of the center of pressure, even in participants who were able to reposition themselves in the wheelchair. The question therefore arises: to what extent are there other parameters that reflect the activity of wheelchair users with the pressure mat? To investigate this, a commercial pressure mat (BodiTrak®) was used to perform the measurements of pressure of 33 adult wheelchair-dependent people with spinal cord injury after 30 and 90 min sitting on the cushion. In addition to the standard output of the pressure mat, graph-based surface analyses (calculation of the area of maximum pressure, calculation of the pressure-loaded measurement area, and pressure-area ratio) was performed retrospectively using Python 3.7. The analysis of the measurements after 30 and 90 min was performed by distinguishing the participants between those who could actively change their position (N = 24) and those who could not (N = 9). The parameters of the pressure mat and the graph-based analyses remained unchanged for active participants. In participants who were unable to actively change their position, the area of maximum pressure and the pressure-area ratio (ratio of maximum pressure area and total pressure-loaded area) increased. Significant differences between minutes 30 and 90 are only found for the pressure-area ratio. Thus, when measuring the seat pressure of wheelchair users, the pressure-area ratio should be taken into account as it reflects the daily relief activities of wheelchair users.
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  • 文章类型: Journal Article
    背景:前路腰椎间融合术(ALIF)和后路脊柱融合术(PSF)在脊柱手术中恢复腰椎前凸中起着关键作用。在单位联合手术和传统的俯卧位PSF之间存在着持续的争论,以优化节段性腰椎前凸。方法:这项回顾性研究分析了59例患者在仰卧位接受ALIF,然后在俯卧位接受PSF。术前测量Cobb角,后ALIF,和使用X射线成像的PSF后。采用单向重复测量ANOVA和具有Bonferroni调整的事后分析来比较不同时间点的平均Cobb角。计算科恩的d效应大小以评估变化的幅度。进行样品大小计算以确保统计能力。结果:平均节段Cobb角从术前(32.2±13.8度)到ALIF后(42.2±14.3度,科恩的d:-0.71,p<0.0001)和PSF后(43.6±14.6度,科恩的d:-0.80,p<0.0001)。ALIF后和PSF后的Cobb角之间没有显着差异(Cohen'sd:-0.10,p=0.14)。当分别分析单螺杆和双螺杆ALIF结构的Cobb角时,结果保持一致。结论:与术前测量相比,仰卧ALIF和俯卧PSF均显着增加了节段性腰椎前凸。ALIF后和PSF后脊柱前凸之间的差异可忽略不计,表明仰卧ALIF后俯卧PSF可能是一种有效的方法。提供手术定位的灵活性,而不影响脊柱前凸的改善。
    Background: Anterior lumbar interbody fusion (ALIF) and posterior spinal fusion (PSF) play pivotal roles in restoring lumbar lordosis in spinal surgery. There is an ongoing debate between combined single-position surgery and traditional prone-position PSF for optimizing segmental lumbar lordosis. Methods: This retrospective study analyzed 59 patients who underwent ALIF in the supine position followed by PSF in the prone position at a single institution. Cobb angles were measured preoperatively, post-ALIF, and post-PSF using X-ray imaging. One-way repeated measures ANOVA and post-hoc analyses with Bonferroni adjustment were employed to compare mean Cobb angles at different time points. Cohen\'s d effect sizes were calculated to assess the magnitude of changes. Sample size calculations were performed to ensure statistical power. Results: The mean segmental Cobb angle significantly increased from preoperative (32.2 ± 13.8 degrees) to post-ALIF (42.2 ± 14.3 degrees, Cohen\'s d: -0.71, p < 0.0001) and post-PSF (43.6 ± 14.6 degrees, Cohen\'s d: -0.80, p < 0.0001). There was no significant difference between Cobb angles after ALIF and after PSF (Cohen\'s d: -0.10, p = 0.14). The findings remained consistent when Cobb angles were analyzed separately for single-screw and double-screw ALIF constructs. Conclusions: Both supine ALIF and prone PSF significantly increased segmental lumbar lordosis compared to preoperative measurements. The negligible difference between post-ALIF and post-PSF lordosis suggests that supine ALIF followed by prone PSF can be an effective approach, providing flexibility in surgical positioning without compromising lordosis improvement.
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  • 文章类型: Journal Article
    背景:头孢唑林可以最大程度地降低青少年特发性脊柱侧凸(AIS)后路脊柱融合术(PSF)后手术部位感染(SSI)的风险。头孢唑啉给药建议各不相同,并且获得的组织浓度的证据有限。方法:我们进行了随机,控制,在PSF治疗AIS期间,通过间歇推注(每3小时30mg/kg)或连续输注(30mg/kg推注,然后每小时10/mg/kg)对12名患者进行了前瞻性药代动力学初步研究。结果:患者在人口统计学和围手术期变量方面匹配良好。虽然总的药物暴露,以曲线下面积(AUC)测量,推注和输注给药的血浆相似,输注剂量在皮下和肌肉组织中获得了更大的头孢唑啉暴露。使用超过最小抑制浓度(MIC)的时间的药效学指标,推注和输注给药均表现良好.然而,当目标杀菌浓度为32µg/mL时,推注组的患者在皮下和肌肉组织中的典型6小时手术时间低于目标的中位数为1/5和1/3,分别。结论:我们得出结论,术中测定头孢唑啉组织浓度是可行的,并且推注和输注头孢唑啉的剂量均达到超过典型MIC的浓度。输注剂量似乎更一致地实现皮下和肌肉组织中的杀菌浓度。
    Background: Cefazolin may minimize the risk of surgical site infection (SSI) following posterior spinal fusion (PSF) for adolescent idiopathic scoliosis (AIS). Cefazolin dosing recommendations vary and there is limited evidence for achieved tissue concentrations. Methods: We performed a randomized, controlled, prospective pharmacokinetic pilot study of 12 patients given cefazolin by either intermittent bolus (30 mg/kg every 3 h) or continuous infusion (30 mg/kg bolus followed by 10/mg/kg per hour) during PSF for AIS. Results: Patients were well matched for demographic and perioperative variables. While total drug exposure, measured as area-under-the-curve (AUC), was similar in plasma for bolus and infusion dosing, infusion dosing achieved greater cefazolin exposure in subcutaneous and muscle tissue. Using the pharmacodynamic metric of time spent above minimal inhibitory concentration (MIC), both bolus and infusion dosing performed well. However, when targeting a bactericidal concentration of 32 µg/mL, patients in the bolus group spent a median of 1/5 and 1/3 of the typical 6 h operative time below target in subcutaneous and muscle tissue, respectively. Conclusions: We conclude that intraoperative determination of cefazolin tissue concentrations is feasible and both bolus and infusion dosing of cefazolin achieve concentrations in excess of typical MICs. Infusion dosing appears to more consistently achieve bactericidal concentrations in subcutaneous and muscle tissues.
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  • 文章类型: Journal Article
    成骨不全症(OI)是由负责胶原蛋白合成的基因或参与胶原纤维形成的多肽的突变引起的遗传性疾病。其主要的骨骼并发症是脊柱侧凸,影响25%至80%的OI患者。OI的脊柱侧弯畸形包括各种畸形,例如鳕鱼,楔状的椎骨或桔梗,颅颈交界区异常,腰骶椎滑脱和滑脱。尽管这些脊柱畸形的确切病理生理学尚不清楚,骨代谢异常与脊柱侧凸曲线的进展有关。骨矿物质密度(BMD)测量表明Z评分显着降低,表明骨质疏松症和脊柱侧凸的进展相关。机械应变增加等因素,关节过度活动,小腿长度差异,骨盆倾斜度,脊髓韧带过度活动,或椎骨微骨折也可能导致脊柱侧凸的严重程度。组织学椎体分析证实了小梁微结构的变化,与骨转换不足有关,表明OI中的全身性骨代谢缺陷。在分子水平上,OI中转化生长因子β(TGFβ)信号的上调可导致骨转换紊乱以及肌肉质量和强度的变化。了解脊柱临床特征与分子通路之间的关系可以揭示TGFβ相关的分子靶点,为OI的新治疗方法铺平了道路。
    Osteogenesis Imperfecta (OI) is a genetic disorder caused by mutations in genes responsible for collagen synthesis or polypeptides involved in the formation of collagen fibers. Its predominant skeletal complication is scoliosis, impacting 25 to 80% of OI patients. Vertebral deformities of the scoliotic curves in OI include a variety of malformations such as codfish, wedged-shaped vertebrae or platyspondyly, craniocervical junction abnormalities, and lumbosacral spondylolysis and spondylolisthesis. Although the precise pathophysiology of these spinal deformities remains unclear, anomalies in bone metabolism have been implicated in the progression of scoliotic curves. Bone Mineral Density (BMD) measurements have demonstrated a significant reduction in the Z-score, indicating osteoporosis and a correlation with the advancement of scoliosis. Factors such as increased mechanical strains, joint hypermobility, lower leg length discrepancy, pelvic obliquity, spinal ligament hypermobility, or vertebrae microfractures may also contribute to the severity of scoliosis. Histological vertebral analysis has confirmed that changes in trabecular microarchitecture, associated with inadequate bone turnover, indicate generalized bone metabolic defects in OI. At the molecular level, the upregulation of Transforming Growth factor-β (TGFβ) signaling in OI can lead to disturbed bone turnover and changes in muscle mass and strength. Understanding the relationship between spinal clinical features and molecular pathways could unveil TGFβ -related molecular targets, paving the way for novel therapeutic approaches in OI.
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  • 文章类型: Journal Article
    脊柱畸形被认为是神经肌肉疾病如脊髓性肌萎缩症(SMA)的重要并发症。SMA患者通常发展为进行性早发性脊柱侧凸,这与功能下降增加有关,不适,和呼吸功能障碍。在二十一世纪的第二个十年,SMA患者的治疗选择发生了很大变化.具体来说,使用药物如nusinersen(Spinraza),阿贝帕沃维奇(Zolgensma),和risdiplam(Evrysdi)极大地改变了SMA患者的景观。这些药物显著改变了运动和呼吸功能,以及脊柱畸形的自然进展。在评估这些药物及其对脊柱侧凸和运动功能发展的影响时,重要的是要考虑开始治疗的时机.在已经出现症状后接受治疗的患者中,已观察到表型向较不严重的亚型转移。这导致不太严重的SMA类型的脊柱侧弯的发作延迟,而严重类型的患者通常无法生存以发展脊柱侧弯的早发性脊柱侧弯增加。在出现症状之前接受治疗的患者几乎可以实现正常的运动功能,并且脊柱侧弯患病率可能显着降低或至少延迟其发作。
    Spinal deformities are considered an important complication of neuromuscular disorders such as spinal muscular atrophy (SMA). SMA patients typically develop progressive early-onset scoliosis, which is associated with increased functional decline, discomfort, and respiratory dysfunction. Over the second decade of the twenty-first century, a lot has changed in terms of the therapeutic options available to people with SMA. Specifically, the use of pharmaceutical agents such as nusinersen (Spinraza), onasemnogene abeparvovec (Zolgensma), and risdiplam (Evrysdi) has dramatically changed the landscape for SMA patients. These medications significantly alter motor- and respiratory functioning, as well as the natural progression of spinal deformities. When evaluating these agents and their impact on the development of scoliosis and motor functioning, it is important to consider the timing of treatment initiation. In patients treated after they had already developed symptoms, a shift of phenotype to a less severe subtype has been observed. This results in a delay in the onset of scoliosis for the less severe SMA types and an increase in early-onset scoliosis for the severe types in patients who would typically not live to develop scoliosis. Patients who receive treatment before they develop symptoms achieve almost normal motor functioning and will likely have a significant decrease in scoliosis prevalence or at least delay its onset.
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  • 文章类型: Journal Article
    青少年特发性脊柱侧凸(AIS)的后路脊柱融合术会导致严重的术后疼痛。胸椎旁阻滞(PVB)在各种手术中提供出色的镇痛效果。我们检查了PVB对接受AIS手术的儿童术后镇痛的影响。在这项研究中,32名计划进行AIS手术的儿童被随机分配接受PVB(PVB组)或不接受阻滞(对照组)。PVB组接受外科医生进行的PVB,每侧0.5mL/kg肾上腺素0.2%罗哌卡因。主要结果是术后6小时休息时的疼痛评分。次要结果包括静息和运动过程中的疼痛评分以及术后48小时的镇痛药使用。术后6h静息疼痛评分在对照组和PVB组之间具有可比性(分别为5.2±2.0和5.1±1.8),没有显著差异。然而,术后1小时,对照组的静息和平均移动疼痛评分明显高于PVB组(p<0.05)。其他时间点的疼痛评分和镇痛药的使用在组间具有可比性。观察到外科医生进行双侧PVB的初始益处,但在术后6小时减少。需要使用各种麻醉剂的未来研究来扩展PVB的效果。
    Posterior spinal fusion for adolescent idiopathic scoliosis (AIS) causes severe postoperative pain. Thoracic paravertebral block (PVB) provides excellent analgesia during various surgeries. We examined the effects of PVB on postoperative analgesia in children undergoing AIS surgery. In this study, 32 children scheduled for AIS surgery were randomly assigned to receive either PVB (PVB group) or no block (control group). The PVB group underwent surgeon-performed PVB with 0.5 mL/kg of adrenalized 0.2% ropivacaine on each side. The primary outcome was the pain score at rest at 6 h postoperatively. Secondary outcomes included pain scores both at rest and during movement and analgesic use for 48 h postoperatively. The postoperative resting pain scores at 6 h were comparable between the control and PVB groups (5.2 ± 2.0 and 5.1 ± 1.8, respectively), with no significant differences. However, at 1 h postoperatively, the control group showed significantly higher resting and mean moving pain scores than the PVB group (p < 0.05). The pain scores at other time points and analgesic use were comparable between the groups. Initial benefits of surgeon-performed bilateral PVB were observed but diminished at 6 h postoperatively. Future research using various anesthetics is needed to extend the effects of PVB.
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  • 文章类型: Journal Article
    背景:Costello综合征(CS)是一种罕见的遗传综合征,由于11号染色体上的HRAS基因突变导致了这种表现,一组特征,如特征性外观,许多先天性缺陷,智力障碍和癌症的遗传易感性,友好的个性,其他人可以识别。CS是非常罕见的,发病率约为1/30万,但属于先天性综合征的最大群体之一,叫做拉丝病,发生在1/1000人的发病率。脊柱侧凸和后凸,以及其他脊柱缺陷,是常见的,在63%和58%的患者中,分别,在成年患者中进行的一项研究表明,75%的患者存在脊柱侧凸;腰椎部分可能存在过度的脊柱前凸和脊柱倒置的弯曲(胸段的前凸和腰椎部分的后凸)。我们研究的目的是提供一例14岁的Costello综合征患者130度严重脊柱侧凸的治疗病例报告,在没有骨骼成熟度的情况下,伴有ChiariII综合征和syrinx。该患者在计划的严重脊柱侧凸手术矫正前3个月接受了大孔减压。患者有资格使用磁控生长棒(MCGR)进行手术治疗。脊柱手术后使用MCGR,在接下来的两年里,我们逐渐进行了MCGR分心;我们进行了最后一次手术,转换为后路脊柱融合术(PSF),同时进行多水平Ponte截骨术,这给了一个很好的和令人满意的手术效果。在围手术期,发生了两种严重的并发症:中央导管引起的气胸和先前未诊断的胃肠道静脉曲张引起的胃肠道出血。这种情况表明,严重和被忽视的脊柱侧弯的治疗是复杂的,需要特殊的准备和与其他合作专家的手术计划。脊柱侧弯从130度矫正到大约48度,矢状平衡明显改善,手术结果非常令人满意,显着改善患者的生活质量和功能。
    BACKGROUND: Costello syndrome (CS) is a rare genetic syndrome in which, due to the occurrence of a mutation in the HRAS gene on chromosome 11 that causes the manifestation, a set of features such as a characteristic appearance, many congenital defects, intellectual disability and a genetic predisposition to cancer, friendly personality, and others can be identified. CS is very rare, with an incidence of ~1/300,000, but it belongs to one of the largest groups of congenital syndromes, called RASopathies, occurring with an incidence of 1/1000 people. Scoliosis and kyphosis, as well as other spinal defects, are common, in 63% and 58% of patients, respectively, and a study conducted among adult patients showed the presence of scoliosis in 75% of patients; there may be excessive lordosis of the lumbar section and inverted curvatures of the spine (lordosis in the thoracic section and kyphosis in the lumbar section). The aim of our study is to present a case report of treatment of severe scoliosis of 130 degrees in a 14-year-old patient with Costello syndrome, with coexisting Chiari II syndrome and syrinx in the absence of skeletal maturity. This patient underwent foramen magnum decompression 3 months before planned surgical correction for severe scoliosis. The patient was qualified for surgical treatment using magnetically controlled growing rods (MCGR). After spine surgery using MCGR, we gradually performed MCGR distraction over the next 2 years; we performed the final surgery, conversion to posterior spinal fusion (PSF) with simultaneous multi-level Ponte osteotomy, which gave a very good and satisfactory surgical result. In the perioperative period, two serious complications occurred: pneumothorax caused by central catheter and gastrointestinal bleeding due to previously undiagnosed gastrointestinal varices. This case shows that the treatment of severe and neglected scoliosis is complicated and requires special preparation and a surgical plan with other cooperating specialists. The scoliosis was corrected from 130 degrees to approximately 48 degrees, sagittal balance was significantly improved, and the surgical outcome was very pleasing, significantly improving quality of life and function for the patient.
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