Musculoskeletal Abnormalities

肌肉骨骼异常
  • 文章类型: Journal Article
    背景:上交叉综合症是一种肌肉失衡和姿势功能障碍的模式,可引起不适和疼痛。这项研究的目的是比较普拉提练习的效果,纠正练习,和亚历山大对13-16岁少女上交叉综合征的技术:一项为期六周的干预研究。
    方法:本研究是准实验,其统计人口包括13至16岁的女学生。有目的地选择45名被诊断患有上交叉综合征的学生作为样本,并随机分配到三组:普拉提练习(N=15),纠正练习(N=15),和亚历山大的技术(N=15)。参与者每节进行60分钟的练习,每周三次,还有六个星期.这项研究的目的是比较普拉提练习的效果,纠正练习,和亚历山大对13-16岁少女上交叉综合征的技术:一项为期六周的干预研究。这项研究于2023-09-19在伊朗临床试验注册中心(IRCT)进行了回顾性注册,以符合该杂志的政策。指定的试验登记号是IRCT20230810059106N1。
    结果:依赖t检验的结果表明,前头角明显减小(p=0.0001),圆肩(p=0.001),和后凸畸形(p=0.0001)作为矫正练习的结果。前头角也显著减小(p=0.0001),圆肩(p=0.002),和后凸畸形(p=0.001)6周后练习亚历山大的技术。然而,在普拉提练习的情况下,前头角显著减小(p=0.110),圆肩(p=0.598),未观察到后凸畸形(p=0.371)。单向方差分析显示,前头角存在显着差异(p=0.012),圆肩(p=0.013),和后凸畸形(p=0.009)。
    结论:亚历山大的技术和矫正练习对前头角的影响,圆形肩部,和后凸畸形几乎相似,比普拉提练习更有效。
    BACKGROUND: Upper Cross Syndrome is a pattern of muscle imbalance and postural dysfunction that can cause discomfort and pain. This study\'s objective was to compare the effects of Pilates exercises, corrective exercises, and Alexander\'s technique on upper cross syndrome in adolescent girls aged 13-16 years: a six-week intervention study.
    METHODS: The present study was Quasi-experimental, and its statistical population consisted of 13 to 16-year-old female students. Forty-five students who were diagnosed with upper cross syndrome were purposefully selected as samples and randomly assigned to three groups: Pilates exercises (N = 15), corrective exercises (N = 15), and Alexander\'s technique (N = 15). The participants performed exercises for 60 min per session, three sessions per week, and six weeks. This study\'s objective was to compare the effects of Pilates exercises, corrective exercises, and Alexander\'s technique on upper cross syndrome in adolescent girls aged 13-16 years: a six-week intervention study. This study was retrospectively registered in the Iranian Registry of Clinical Trials (IRCT) on 2023-09-19 to comply with the journal\'s policies. The assigned trial registration number is IRCT20230810059106N1.
    RESULTS: The results of the dependent t-test showed significant decreases in forward head angle (p = 0.0001), rounded shoulder (p = 0.001), and kyphosis (p = 0.0001) as a result of corrective exercises. There were also significant decreases in forward head angle (p = 0.0001), rounded shoulder (p = 0.002), and kyphosis (p = 0.001) after six weeks of practising Alexander\'s technique. However, in the case of Pilates exercises, a significant decrease in forward head angle (p = 0.110), rounded shoulder (p = 0.598), and kyphosis (p = 0.371) was not observed. The one-way analysis of variance revealed a significant difference in the forward head angle (p = 0.012), rounded shoulders (p = 0.013), and kyphosis (p = 0.009).
    CONCLUSIONS: The effect of Alexander\'s technique and corrective exercises on forward head angle, rounded shoulder, and kyphosis abnormalities was almost similar and more effective than pilates exercises.
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  • 文章类型: Journal Article
    目的:肌肉骨骼疾病(MSD)对所有年龄段的人都有重大影响,专业,和来自世界各地的领域,对生活质量和整体健康结果产生负面影响。该研究旨在调查沙特阿拉伯物理治疗师中倦怠综合征(BS)与MSD之间的关联。
    方法:进行了横断面研究。该研究包括性别,没有已知合并症,没有最近受伤和事故的物理治疗师。哥本哈根倦怠清单用于确定倦怠水平。使用标准化北欧问卷(NMQ)分析肌肉骨骼疾病。使用卡方检验来观察两个分类变量之间的关联,并且P值<0.05被认为是统计学上显著的。
    结果:总共招募了133名参与者,观察到70名(53%)参与者的倦怠水平较低(BoL)。据透露,65岁(49%)的人颈部出现问题,并前往医生诊所就诊,在过去的12个月中,有47人(35%)的下背部有问题,在过去的12个月中,有60名(45%)的上背部有问题,无法正常活动,而在过去的7天中,有13名(9.8%)的参与者的肩膀有问题。慢性病史,教育,练习设置,吸烟状况与倦怠水平有统计学意义。上背部有麻烦,弯头,手腕/手,臀部/大腿,脚踝/脚,和颈部也被发现是重要的。
    结论:结果显示,一些人口统计学因素,如教育,练习设置,吸烟状况,慢性病史与BS显著相关。此外,据观察,MSD对BoL有显著影响。
    OBJECTIVE: There is a significant impact of musculoskeletal disorders (MSDs) on individuals of all ages, professions, and fields from various countries worldwide, leading to a negative impact on quality of life and overall health outcomes. The study was conducted to investigate the association between Burnout syndrome (BS) and MSDs among physiotherapists in Saudi Arabia.
    METHODS: A cross-sectional study was carried out. Physiotherapists of either gender and with no known co-morbidities and who were not suffering from recent injuries and accidents were included in the study. The Copenhagen Burnout Inventory was used to determine the level of burnout. Musculoskeletal disorders were analyzed using the Standardized Nordic Questionnaire (NMQ). The chi-square test was used to observe the association between two categorical variables and a p-value <0.05 was considered to be statistically significant.
    RESULTS: A total of 133 participants were recruited and it was observed that 70 (53%) participants had low burnout levels (BoL). It was revealed that 65 (49%) had trouble in the neck and visited a physician clinic, 47 (35%) had trouble in the lower back during the last 12 months, 60 (45%) had trouble in the upper back during the last 12 months that prevented the normal activity and 13 (9.8%) participants had trouble in the shoulders during the last 7 days. History of chronic disease, education, practice setting, and smoking status with Burnout level was statistically significant. Trouble in the upper back, elbow, wrist/hands, hips/thighs, ankle/feet, and neck were also found to be significant.
    CONCLUSIONS: The outcomes revealed that a few demographic factors such as education, practice setting, smoking status, and chronic disease history were significantly associated with BS. Furthermore, it was observed that MSD had a significant impact on BoL.
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  • 文章类型: Journal Article
    保守治疗的海绵状畸形(CM)患者的出血风险和长期预后知识较差。在这项工作中,我们研究了10年期间CM相关出血的发生情况,并调查了出血的危险因素.我们的机构数据库筛选了2003年至2021年间收治的大脑(CCM)或髓内脊髓(ISCM)CM患者。排除接受手术的患者和未完成随访的患者。进行分析以确定危险因素并确定出血的累积风险。共纳入91例CM患者。调整后的多变量逻辑回归分析将诊断时的出血(p=0.039)和CM定位到脊柱(p=0.010)确定为(再)出血的预测因子。通过Cox回归分析,这两个危险因素仍然是独立的预测因子(p=0.049;p=0.016)。整个队列的累积10年出血风险为30%,39%用于诊断出血的患者,67%用于ISCM。在未经治疗的10年随访中,出血的概率随着时间的推移而增加,特别是在出现出血和脊髓定位的情况下。这种增加的强度可能会随着时间的推移而下降,但仍然相当高。这些发现可能表明ISCM患者的病程相当激进,并且可能支持早期手术治疗。
    Knowledge of the bleeding risk and the long-term outcome of conservatively treated patients with cavernous malformations (CM) is poor. In this work, we studied the occurrence of CM-associated hemorrhage over a 10-year period and investigated risk factors for bleeding. Our institutional database was screened for patients with cerebral (CCM) or intramedullary spinal cord (ISCM) CM admitted between 2003 and 2021. Patients who underwent surgery and patients without completed follow-up were excluded. Analyses were performed to identify risk factors and to determine the cumulative risk for hemorrhage. A total of 91 CM patients were included. Adjusted multivariate logistic regression analysis identified bleeding at diagnosis (p = 0.039) and CM localization to the spine (p = 0.010) as predictors for (re)hemorrhage. Both risk factors remained independent predictors through Cox regression analysis (p = 0.049; p = 0.016). The cumulative 10-year risk of bleeding was 30% for the whole cohort, 39% for patients with bleeding at diagnosis and 67% for ISCM. During an untreated 10-year follow-up, the probability of hemorrhage increased over time, especially in cases with bleeding at presentation and spinal cord localization. The intensity of such increase may decline throughout time but remains considerably high. These findings may indicate a rather aggressive course in patients with ISCM and may endorse early surgical treatment.
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  • 文章类型: Journal Article
    目的:评估Lenke5C青少年特发性脊柱侧凸(AIS)腰骶段移行椎骨(LSTV)的发生率,并评估当最低器械椎骨(LIV)固定在L3时,术后结局与LSTV之间的关系。
    方法:该研究包括61例Lenke5CAIS患者,这些患者接受了L3作为LIV的融合手术,随访至少5年。患者分为两组:LSTV+和LSTV-。人口统计,外科,和射线照相数据,包括L4倾斜和胸腰椎/腰椎(TL/L)Cobb角,获得并分析。
    结果:在15例患者(24.5%)中观察到LSTV。术前两组的L4倾斜无显著差异(P=0.54);LSTV组术后明显高于LSTV组(2周:LSTV+=11.7±3.1,LSTV-=8.8±3.2,P=0.013;2年:LSTV+=11.5±3.5,LSTV-=7.9±4.1,P=0.006;5年:LSTV+=9.8±3.1,LSTV-=7.3±4.5,P=0.042)。LSTV+组术后TL/L曲线较大,术后2周和2年差异有统计学意义(术前:LSTV=53.5±11.2,LSTV-=51.7±10.3,P=0.675;2周:LSTV=16.1±5.0,LSTV-=12.2±6.6,P=0.027;2年:LSTV-=21.7±5.9,LSTV-=17.6±5.9,5年=LTV=18.35±5.8
    结论:Lenke5CAIS患者中LSTV的患病率为24.5%。Lenke5CAIS患者LSTV的L3LIV患者的术后L4倾斜明显大于没有LSTV的患者,并保留了TL/L曲线。
    To assess the incidence of lumbosacral transitional vertebra (LSTV) in Lenke 5C adolescent idiopathic scoliosis (AIS) and evaluate the relationship between postoperative outcomes and LSTV when the lowest instrumented vertebra (LIV) is fixed at L3.
    The study included 61 patients with Lenke 5C AIS who underwent fusion surgery of L3 as the LIV who were followed-up for a minimum of 5 years. Patients were divided into two groups: LSTV + and LSTV-. Demographic, surgical, and radiographic data, including L4 tilt and thoracolumbar/lumbar (TL/L) Cobb angle, were obtained and analyzed.
    LSTV was observed in 15 patients (24.5%). The L4 tilt was not significantly different between the two groups preoperatively (P = 0.54); however, it was significantly greater in the LSTV group postoperatively (2 weeks: LSTV +  = 11.7 ± 3.1, LSTV -  = 8.8 ± 3.2, P = 0.013; 2 years: LSTV +  = 11.5 ± 3.5, LSTV -  = 7.9 ± 4.1, P = 0.006; 5 years: LSTV +  = 9.8 ± 3.1, LSTV -  = 7.3 ± 4.5,  P= 0.042). The postoperative TL/L curve was greater in the LSTV + group, with significant differences at 2 weeks and 2 years postoperatively (preoperative: LSTV +  = 53.5 ± 11.2, LSTV -  = 51.7 ± 10.3,P = 0.675; 2 weeks: LSTV +  = 16.1 ± 5.0, LSTV- = 12.2 ± 6.6, P = 0.027; 2 years: LSTV +  = 21.7 ± 5.9, LSTV -  = 17.6 ± 5.9, P = 0.035; 5 years: LSTV +  = 18.7 ± 5.8, LSTV -  = 17.0 ± 6.1, P = 0.205).
    The prevalence of LSTV in Lenke 5C AIS patients was 24.5%. Lenke 5C AIS patients with LSTV with the LIV at L3 had a significantly greater postoperative L4 tilt than those without LSTV and retained the TL/L curve.
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  • 文章类型: Journal Article
    目的:研究用关节位置感(JPS)测量的慢性颈部和下背痛患者的本体感觉准确性是否仅在患处或远处受损,不受疼痛影响。
    方法:横断面研究。
    方法:治疗背部和颈部疼痛的跨学科门诊康复诊所。
    方法:慢性颈痛患者(n=30),慢性腰背痛患者(n=30),以及年龄和性别匹配的无症状对照受试者(n=30;N=90)。
    方法:不适用。
    方法:患者和无症状对照受试者完成了颈椎JPS的测试程序,腰椎,和脚踝按随机顺序。使用Pearson相关系数对JPS与临床特征的单变量方差分析和关联进行组间差异分析。
    结果:在颈椎JPS中,慢性颈痛患者(P<.001)和慢性下腰痛患者(P<.01)均与无症状对照组显着不同,腰椎和踝关节,不管痛苦的地方。患者组之间无差异(P>.05)。JPS与临床特征的关联,然而,无法显示。
    结论:这些结果表明,慢性和下腰痛患者的本体感觉准确性普遍受损,并且中枢感觉运动过程在肌肉骨骼疼痛中的作用。
    To investigate whether proprioceptive accuracy measured with the Joint Position Sense (JPS) in patients with chronic neck and low back pain is impaired exclusively in affected areas or also in distant areas, not affected by pain.
    Cross-sectional study.
    Interdisciplinary outpatient rehabilitation clinic for back and neck pain.
    Patients with chronic neck pain (n=30), patients with chronic low back pain (n=30), and age- and sex-matched asymptomatic control subjects (n=30; N=90).
    Not applicable.
    Patients and asymptomatic control subjects completed a test procedure for the JPS of the cervical spine, lumbar spine, and ankle in a randomized order. Between group differences were analyzed with the univariate analysis of variance and associations of the JPS with clinical features using the Pearson\'s correlation coefficient.
    Both patients with chronic neck pain (P<.001) and patients with chronic low back pain (P<.01) differed significantly from asymptomatic controls in the JPS of the cervical spine, lumbar spine and ankle joint, regardless of the painful area. No difference was shown between patient groups (P>.05). An association of the JPS with clinical characteristics, however, could not be shown.
    These results suggest widespread impairment of proprioceptive accuracy in patients with chronic and low back pain and a role for central sensorimotor processes in musculoskeletal pain conditions.
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  • 文章类型: Observational Study
    目的:脊柱海绵状畸形(SCM)的临床结局因其自然史不明确而有所不同,SCM患者可靠的预后预测模型有限。本研究的目的是调查预测SCM术后患者一年神经状况的潜在因素。
    方法:这是一项针对SCMs连续患者的多中心前瞻性观察性研究,纳入2015年1月至2021年1月间进行显微外科治疗的SCMs。结果定义为术后一年的美国脊髓损伤协会损害量表(AIS)等级。多变量分析用于构建患者预后的最佳预测模型。
    结果:我们确定了268名符合条件的SCM患者。一年时,51例患者(19.0%)的神经系统转归较术前基线恶化。在多变量逻辑回归中,不良结局的最佳预测模型包括症状持续时间≥26个月(95%CI2.80-16.96,P<0.001),尺寸≤5mm(95%CI1.43-13.50,P=0.010),完整髓内(95%CI1.69-8.14,P=0.001),蛛网膜下腔出血(95%CI2.92-12.57,P<0.001),AISB(95%CI1.91-40.93,P=0.005)和AISC(95%CI1.12-14.54,P=0.033)。
    结论:病变的入院大小,形态学,症状持续时间,AIS分级和蛛网膜下腔出血的存在是预测SCMs术后神经系统预后的重要预后因素。通过对所有可能影响预后的因素的系统分析,应证明手术切除有症状的SCM的决定是合理的。
    Clinical outcome of spinal cavernous malformation (SCM) varies because of its unclear natural history, and reliable prognostic prediction model for SCM patients is limited. The aim of the present study was to investigate potential factors that predict one-year neurological status in postoperative patients with SCM.
    This was a multicenter prospective observational study in consecutive patients with SCMs. SCMs treated microsurgically between January 2015 and January 2021 were included. Outcome was defined as the American Spinal Injury Association Impairment Scale (AIS) grade at one year after operation. Multivariable analyses were used to construct the best predictive model for patient outcomes.
    We identified 268 eligible SCM patients. Neurological outcome had worsened from preoperative baseline in 51 patients (19.0%) at one year. In the multivariable logistic regression, the best predictive model for unfavorable outcome included symptom duration ≥ 26 months (95% CI 2.80-16.96, P < 0.001), size ≤ 5 mm (95% CI 1.43-13.50, P = 0.010), complete intramedullary (95% CI 1.69-8.14, P = 0.001), subarachnoid hemorrhage (95% CI 2.92-12.57, P < 0.001), AIS B (95% CI 1.91-40.93, P = 0.005) and AIS C (95% CI 1.12-14.54, P = 0.033).
    Admission size of the lesion, morphology, symptom duration, AIS grade and the presence of subarachnoid hemorrhage were strong outcome predictors regarding prognostication of neurological outcome in postoperative patients with SCMs. A decision to surgically remove a symptomatic SCM should be justified by systematic analysis of all factors potentially affecting outcome.
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  • 文章类型: Journal Article
    为了最大程度地减少手术并发症和分阶段手术,经常在畸形矫正期间使用光环牵引。但使用晕轮牵引治疗继发感染的难治性宫颈后凸畸形从未见报道。这项研究调查了晕轮牵引在治疗与难治性脊柱后凸相关的宫颈感染患者中的作用。我们回顾性分析了在脊柱科接受治疗的48例宫颈感染伴难治性脊柱后凸的患者。患者分为两组,牵引组(A)和非牵引组(B)。A组接受术前光环牵引,然后手术,B组仅接受手术治疗。在两组之间,我们分析了后凸畸形的矫正,融合的水平,红细胞沉降率(ESR),C反应蛋白(CRP),颈部残疾指数(NDI)评分的功能改善,和并发症。A组后凸畸形的矫正效果优于B组(27.01±11.54)0比(18.08±10.04)0(P=0.01,Z=-2.44)。两组在功能改善方面无统计学差异,融合的水平,ESR和CRP。B组3例翻修手术。术前晕轮牵引后手术治疗后凸畸形在治疗宫颈感染伴难治性后凸畸形患者中的优势。
    To minimize surgical complications and staged procedures halo-traction is often used during deformity corrections. But the use of halo-traction in the treatment of refractory cervical kyphosis secondary to infections has never been reported. This study investigated the role of halo-traction in the treatment of cervical infection patients associated with refractory kyphosis. We retrospectively reviewed 48 patients with cervical infection associated with refractory kyphosis who were treated in our spine department. Patients were divided into two groups, the traction group (A) and the non-traction group (B). Group A underwent preoperative halo-traction followed by surgery, while group B underwent surgery alone. Between the two groups, we analyzed the kyphosis deformity correction, level of fusions, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), functional improvement by Neck disability index (NDI) score, and complications. Group A had a better correction of kyphosis deformity compared to group B (27.01 ± 11.54)0 versus (18.08 ± 10.04)0 (P = 0.01, Z =  - 2.44). No statistically significant differences between the two groups in terms of functional improvement, level of fusions, ESR and CRP. Group B had 3 revision surgery cases. Preoperative halo-traction followed by surgery is superior in kyphosis correction in the treatment of patients with cervical infections with refractory kyphosis.
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  • 文章类型: Journal Article
    建立老年慢性骨质疏松性椎体骨折(COVF)后路内固定术后近端交界性脊柱后凸(PJK)预测评分系统。
    回顾性分析2013年1月至2017年12月在我院确诊为COVF并接受后路内固定术的88例患者的病历资料。根据患者术后是否发生PJK分为两组,即,PJK组25例,非PJK组63例。记录并分析了以下临床特征:年龄,性别,体重指数(BMI),骨矿物质密度(BMD),吸烟史,骨折段,近端连接角度,矢状椎体轴,骨盆发病率(PI)-腰椎前凸(LL),骨盆倾斜(PT),骶骨斜坡(SS),后韧带复合体(PLC)损伤,上器械椎骨,下部器械椎骨,和固定段的数量。评估了PJK组中这些临床特征的患病率,并采用logistic回归分析建立评分体系。评分系统的性能也得到了前瞻性验证。
    根据被证实为PJK的重要预测因子的五个临床特征,建立了预测评分系统。即,年龄>70岁,BMI>28kg/m2,BMD<-3.5SD,术前PI-LL>20°,PLC损伤。PJK的得分明显高于非PJK(7.80分vs.2.83分,t=9.556,P<0.001),评分系统的最佳临界值为5分。评分系统对术后PJK预测的敏感性和特异性分别为80.00%和88.89%,分别,在推导集中,在验证集中为75.00%和80.00%。
    预测评分系统对老年COVF患者后路内固定术后PJK的预测具有满意的敏感性和特异性。6-11分的患者术后发生PJK的风险较高,而0-5的分数很低。
    To establish a predictive scoring system for proximal junctional kyphosis (PJK) after posterior internal fixation in elderly patients with chronic osteoporotic vertebral fracture (COVF).
    The medical records of 88 patients who were diagnosed with COVF and underwent posterior internal fixation in our hospital from January 2013 to December 2017 were retrospectively analyzed. The included patients were divided into two groups according to whether they suffered PJK after surgery, namely, the PJK group (25 cases) and non-PJK group (63 cases). The following clinical characteristics were recorded and analyzed: age, gender, body mass index (BMI), bone mineral density (BMD), smoking history, fracture segment, proximal junction angle, sagittal vertebral axis, pelvic incidence (PI)-lumbar lordosis (LL), pelvic tilt (PT), sacral slope (SS), posterior ligamentous complex (PLC) injury, upper instrumented vertebra, lower instrumented vertebra, and the number of fixed segments. The prevalence of these clinical characteristics in the PJK group was evaluated, and the scoring system was established using logistic regression analysis. The performance of the scoring system was also prospectively validated.
    The predictive scoring system was established based on five clinical characteristics confirmed as significant predictors of PJK, namely, age > 70 years, BMI > 28 kg/m2, BMD < -3.5 SD, preoperative PI-LL > 20°, and PLC injury. PJK showed a significantly higher score than non-PJK (7.80 points vs. 2.83 points, t=9.556, P<0.001), and the optimal cutoff value for the scoring system was 5 points. The sensitivity and specificity of the scoring system for predicting postoperative PJK were 80.00% and 88.89%, respectively, in the derivation set and 75.00% and 80.00% in the validation set.
    The predictive scoring system was confirmed with satisfactory sensitivity and specificity in predicting PJK after posterior internal fixation in elderly COVF patients. The risk of postoperative PJK in patients with a score of 6-11 is high, while the score of 0-5 is low.
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  • 文章类型: Journal Article
    马凡氏综合征与肌肉骨骼系统异常有关,包括脊柱侧凸,胸部畸形,前髋臼,和足部畸形。在一生中,许多患有马凡氏综合征的患者将需要治疗;然而,一生中的肌肉骨骼发病率没有得到很好的描述。本研究的目的是评估马凡氏综合征患者肌肉骨骼疾病的总体负担。
    基于注册表的,1977年至2014年对根特Ⅱ型确诊为Marfan综合征的患者进行的全国性流行病学研究.每个病人的年龄都是匹配的,以及背景人群中多达100个对照的性别。
    我们确定了407例马凡氏综合征患者和40,700例对照,并使用Cox比例风险比(HR)比较了他们的肌肉骨骼诊断和手术治疗。与对照组相比,马凡氏综合征患者注册肌肉骨骼诊断的风险显着增加(HR:1.94(1.69-2.24)。六分之一的马凡氏综合症患者患有脊柱侧弯(HR:36.7(27.5-48.9)。与男性相比,脊柱侧凸在患有马凡氏综合征的女性中更为常见(HR:4.30(1.73-1.08))。11人中有1人患有胸肌畸形HR:40.8(28.1-59.3),六分之一的脚畸形。主要是平面(HR:26.0(15.2-44.3)。接受肌肉骨骼手术的马凡氏综合征(94/407)患者比例也显着较高(HR:1.76(1.43-2.16))。手术的主要区域是脊柱,错误校正,和脚/脚踝的手术。10例马凡氏综合征患者接受了择期骨科手术,直到晚年才被识别和诊断为马凡氏综合征。这些人都没有脊柱侧弯,胸部畸形或足部畸形。主动脉夹层患者中,在至少有一个主要肌肉骨骼异常的患者中,解剖年龄为34.3岁。在没有重大异常的患者中,解剖年龄为45.1岁(p<0.01)。
    肌肉骨骼疾病的程度在马凡氏综合征中相当显著,许多人在他们的一生中需要矫正手术。在治疗具有马凡氏综合征样表型的患者时,外科医生应注意未确诊的马凡氏综合征患者。
    Marfan syndrome is associated with abnormalities in the musculoskeletal system including scoliosis, pectus deformities, protrusio acetabuli, and foot deformities. Over a life span, many patients with Marfan syndrome will need treatment; however, the musculoskeletal morbidity over a life span is not well described. The aim of the present study was to assess the overall burden of musculoskeletal disease in patients with Marfan syndrome.
    A registry-based, nationwide epidemiological study of patients with a Ghent II verified Marfan syndrome diagnosis from 1977 to 2014. Each patient was matched on age, and sex with up to 100 controls from the background population.
    We identified 407 patients with Marfan syndrome and 40,700 controls and compared their musculoskeletal diagnoses and surgical treatments using Cox proportional hazards ratio (HR). The risk of a registration of a musculoskeletal diagnosis in patients with Marfan syndrome was significantly increased compared to controls (HR: 1.94 (1.69-2.24). One out of six with Marfan syndrome was registered with scoliosis (HR: 36.7 (27.5-48.9). Scoliosis was more common in women with Marfan syndrome compared to men (HR: 4.30 (1.73-1.08)). One out of 11 were registered with a pectus deformity HR: 40.8 (28.1-59.3), and one out of six with a deformity of the foot. Primarily pes planus (HR: 26.0 (15.2-44.3). The proportion of patients with Marfan syndrome (94/407) that underwent musculoskeletal surgery was also significantly higher (HR: 1.76 (1.43-2.16)). The major areas of surgery were the spine, pectups correction, and surgery of the foot/ankle. Ten patients with Marfan syndrome had elective orthopedic surgery without being recognized and diagnosed with Marfan syndrome until later in life. None of these had scoliosis, pectus deformity or a foot deformity. Among patients with an aortic dissection, the age at dissection was 34.3 years in those with at least one major musculoskeletal abnormality. In patients without a major abnormality the age at dissection was 45.1 years (p < 0.01).
    The extent of musculoskeletal disease is quite significant in Marfan syndrome, and many will need corrective surgery during their life span. Surgeons should be aware of undiagnosed patients with Marfan syndrome when treating patients with a Marfan syndrome like-phenotype.
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  • 文章类型: Journal Article
    锻炼不容忍,心力衰竭(HF)患者的标志,与肌肉无力有关.然而,在射血分数保留或降低(HFpEF或HFrEF)表型的患者中,其致病微循环和肌肉特征尚不清楚.可能导致外周微循环受损的肌肉骨骼异常是HF的肌肉减少症和肌肉力量降低,这意味着降低氧化能力和灌注会影响运动过程中的运输和氧利用,微血管肌肉功能的一项重要任务。除此之外,在同样存在骨骼肌肌病的HF患者中,骨骼肌微循环异常也与运动不耐受有关。这项横断面研究旨在比较等速肌力测试过程中通过近红外光谱(NIRS)响应的肌肉微循环动力学和超声得出的参数(回声强度为股直肌,而在具有HFpEF和HFrEF表型和不同功能严重度(WeberA级,B,andC).评估了28例年龄匹配的HFpEF(n=16)和HFrEF(n=12)患者。我们发现具有WeberC严重程度的患者之间的表型差异,HFrEF患者的氧合血红蛋白降低(O2Hb,μM)(-10.9±3.8vs.-23.7±5.7,p=0.029)在运动过程中,而HFpEF在恢复期达到较低的O2Hb(-3.0±3.4vs.5.9±2.8,p=0.007)。WeberC级HFpEF的回声强度也高于HFrEF患者(29.7±8.4vs.超声衍生变量中的15.1±6.8,p=0.017)。我们的初步研究显示,HFpEF与HFpEF的局部微循环动力学受损更为明显。HFrEF患者在进行肌肉力量锻炼时,结合通过超声成像检测到的肌肉骨骼异常,这可能有助于解释在HFpEF患者中常见的运动不耐受。
    Exercise intolerance, a hallmark of patients with heart failure (HF), is associated with muscle weakness. However, its causative microcirculatory and muscle characteristics among those with preserved or reduced ejection fraction (HFpEF or HFrEF) phenotype is unclear. The musculoskeletal abnormalities that could result in impaired peripheral microcirculation are sarcopenia and muscle strength reduction in HF, implying lowered oxidative capacity and perfusion affect transport and oxygen utilization during exercise, an essential task from the microvascular muscle function. Besides that, skeletal muscle microcirculatory abnormalities have also been associated with exercise intolerance in HF patients who also present skeletal muscle myopathy. This cross-sectional study aimed to compare the muscle microcirculation dynamics via near-infrared spectroscopy (NIRS) response during an isokinetic muscle strength test and ultrasound-derived parameters (echo intensity was rectus femoris muscle, while the muscle thickness parameter was measured on rectus femoris and quadriceps femoris) in heart failure patients with HFpEF and HFrEF phenotypes and different functional severities (Weber Class A, B, and C). Twenty-eight aged-matched patients with HFpEF (n = 16) and HFrEF (n = 12) were assessed. We found phenotype differences among those with Weber C severity, with HFrEF patients reaching lower oxyhemoglobin (O2Hb, μM) (-10.9 ± 3.8 vs. -23.7 ± 5.7, p = 0.029) during exercise, while HFpEF reached lower O2Hb during the recovery period (-3.0 ± 3.4 vs. 5.9 ± 2.8, p = 0.007). HFpEF with Weber Class C also presented a higher echo intensity than HFrEF patients (29.7 ± 8.4 vs. 15.1 ± 6.8, p = 0.017) among the ultrasound-derived variables. Our preliminary study revealed more pronounced impairments in local microcirculatory dynamics in HFpEF vs. HFrEF patients during a muscle strength exercise, combined with muscle-skeletal abnormalities detected via ultrasound imaging, which may help explain the commonly observed exercise intolerance in HFpEF patients.
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