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71.
Clinical Spectrum of Capillary Malformation–Arteriovenous Malformation Syndrome Presenting to a Pediatric Dermatology Practice: A Retrospective Study 下载免费PDF全文
Nicole A. Weitz M.D. Christine T. Lauren M.D. Gerald G. Behr M.D. June K. Wu M.D. Jessica J. Kandel M.D. Philip M. Meyers M.D. Sally Sultan M.D. Kwame Anyane‐Yeboa M.D. Kimberly D. Morel M.D. Maria C. Garzon M.D. 《Pediatric dermatology》2015,32(1):76-84
Capillary malformation–arteriovenous malformation syndrome (CM‐AVM) is an autosomal dominant disorder caused by RASA1 mutations. The prevalence and phenotypic spectrum are unknown. Evaluation of patients with multiple CMs is challenging because associated AVMs can be life threatening. The objective of this study was to describe the clinical characteristics of children presenting with features of CM‐AVM to an academic pediatric dermatology practice. After institutional review board approval was received, a retrospective chart review was performed of patients presenting between 2009 and 2012 with features of CM‐AVM. We report nine cases. Presenting symptoms ranged from extensive vascular stains and cardiac failure to CMs noted incidentally during routine skin examination. All demonstrated multiple CMs, two had Parkes Weber syndrome, and two had multiple infantile hemangiomas. Seven patients had family histories of multiple CMs; three had family histories of large, atypical CMs. Six had personal or family histories of AVMs. Genetic evaluation was recommended for all and was pursued by six families; four RASA1 mutations were identified, including one de novo. Consultations with neurology, cardiology, and orthopedics were recommended. Most patients (89%) have not required treatment to date. CM‐AVM is an underrecognized condition with a wide clinical spectrum that often presents in childhood. Further evaluation may be indicated in patients with multiple CMs. This study is limited by its small and retrospective nature. 相似文献
72.
目的 观察“调任通督法”针刺治疗围绝经期潮热的临床疗效和安全性。方法 将符合纳入标准的72例围绝经期潮热患者,按照1∶1的比例随机分为治疗组(针刺组)和对照组(等待组)。治疗组取穴百会、印堂、神庭、关元、气海、中脘、阴郄、太溪、复溜、三阴交。针刺治疗每次持续30 min,每周2次,连续治疗6周。对照组为等待组,试验期间仅给予同针刺组相同的健康宣教,两组分别于治疗前、治疗第3周、治疗第6周以及第10周随访评估其潮热频率和程度,以及更年期生活质量量表(Menopause-specific quality of life questionnaire,Men-QoL)的变化情况,同时记录针刺不良事件的发生。结果 ①潮热频率及潮热程度:在治疗后及随访时,针刺组较等待组有明显改善,差异具有统计学意义(P < 0.01)。与治疗前相比,针刺组第3周即出现了潮热频率明显下降的趋势(P < 0.01),治疗后以及随访期针刺组潮热频率及潮热程度均较治疗前有明显统计学差异(P < 0.01)。而等待组在各时间点潮热频率及潮热程度均无统计学差异(均P > 0.05)。②围绝经期生活质量量表:治疗后针刺组在Men-QoL总分、血管舒缩功能评分、社会心理、体能状况较等待组比较均有统计学差异(均P < 0.01)。在随访期,针刺组在Men-QoL社会心理以及体能方面明显改善,差异均具有统计学意义(P < 0.01,P < 0.05)。两组Men-QoL性功能在治疗后及随访期均无统计学差异(P > 0.05)。与治疗前相比,在治疗后及随访期,针刺组Men-QoL总分、血管舒缩功能、社会心理、体能状况均有统计学差异(均P < 0.05),在Men-QoL性功能方面无统计学差异(P > 0.05)。等待组在Men-QoL总分及四个域方面均无明显改善,无统计学意义(均P > 0.05)。结论 调任通督法针刺治疗围绝经期潮热安全有效,且对围绝经期生活质量有所改善。 相似文献
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Kui Li Jiawen Wang Shengxiong Liu Sen Su Chenjian Feng Xiaoxiang Fan Zhiyong Yin 《中华创伤杂志(英文版)》2015,18(2):65-73
Objective: To study the biomechanical mechanism of head injuries beaten with sticks, which is common in the battery or assaultive cases.
Methods: In this study, the Hybrid-III anthropomorphic test device and finite element model (FEM) of the total human model for safety (THUMS) head were used to determine the biomechanical response of head while being beaten with different sticks. Total eight Hybrid-III tests and four finite element simulations were conducted. The contact force, resultant acceleration of head center of gravity, intracranial pressure and von Mises stress were calculated to determine the different biomechanical behavior of head with beaten by different sticks.
Results: In Hybrid-III tests, the stick in each group demonstrated the similar kinematic behavior under the same loading condition. The peak values of the resultant acceleration for thick iron stick group, thin iron stick group, thick wooden stick group and thin wooden stick group were 203.4 g, 221.1 g, 170.5 g and 122.2 g respectively. In finite element simulations, positive intracranial pressure was initially observed in the frontal comparing with negative intracranial pressure in the contra-coup site. Subsequently the intracranial pressure in the coup site was decreasing toward negative value while the contra-coup intracranial pressure increasing toward positive values.
Conclusions: The results illustrated that the stiffer and larger the stick was, the higher the von Mises stress, contact force and intracranial pressure were. We believed that the results in the Hybrid-III tests and THUMS head simulations for brain injury beaten with sticks could be reliable and useful for better understanding the injury mechanism. 相似文献
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77.
目的 探讨血清谷丙转氨酶(ALT)与新诊2型糖尿病(T2DM)患病风险之间的关联,为T2DM的防治提供科学依据。方法 采用4阶段分层随机抽样的方法选取2006年和2009年参与青岛糖尿病预防项目的研究对象男性3 012例、女性4 422例,采用Pearson相关检验分析不同性别ALT与FPG、2 h PG的相关性,并利用多因素logistic回归分析ALT与新诊T2DM患病的关系。结果 男性中,ALT与空腹血浆血糖(FPG)、餐后2 h血浆血糖(2 h PG)的Pearson相关系数分别为0.088、0.080(均P<0.01);多因素logistic回归显示,在调整了年龄、BMI、糖尿病家族史、城乡、教育、婚姻、收入、吸烟及饮酒状况等混杂因素后,ALT的第4分位(Q4)组新诊T2DM患病风险是第1分位(Q1)组的1.832倍(OR = 1.832,95% CI:1.324~2.534,P<0.01)。女性中,ALT与FPG、2h PG的Pearson相关系数分别为0.065、0.108(均P<0.01);多因素logistic回归显示,在调整了年龄、BMI、糖尿病家族史、城乡、教育、婚姻、收入、吸烟及饮酒状况等混杂因素后,ALT的第4分位(Q4)组新诊T2DM患病风险是第1分位(Q1)组的1.445倍(OR = 1.445,95% CI:1.087~1.919,P<0.05)。结论 在男、女性人群中,ALT水平升高与T2DM患病相关,且这种相关性不受年龄、BMI、糖尿病家族史等的影响。 相似文献
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79.
Xue Yao Yan Zhang Jian Hao Hui-Quan Duan Chen-Xi Zhao Chao Sun Bo Li Bao-You Fan Xu Wang Wen-Xiang Li Xuan-Hao Fu Yong Hu Chang Liu Xiao-Hong Kong Shi-Qing Feng 《中国神经再生研究》2019,(3)
Ferroptosis is an iron-dependent novel cell death pathway. Deferoxamine, a ferroptosis inhibitor, has been reported to promote spinal cord injury repair. It has yet to be clarified whether ferroptosis inhibition represents the mechanism of action of Deferoxamine on spinal cord injury recovery. A rat model of Deferoxamine at thoracic 10 segment was established using a modified Allen's method. Ninety 8-week-old female Wistar rats were used. Rats in the Deferoxamine group were intraperitoneally injected with 100 mg/kg Deferoxamine 30 minutes before injury. Simultaneously, the Sham and Deferoxamine groups served as controls. Drug administration was conducted for 7 consecutive days. The results were as follows:(1) Electron microscopy revealed shrunken mitochondria in the spinal cord injury group.(2) The Basso, Beattie and Bresnahan locomotor rating score showed that recovery of the hindlimb was remarkably better in the Deferoxamine group than in the spinal cord injury group.(3) The iron concentration was lower in the Deferoxamine group than in the spinal cord injury group after injury.(4) Western blot assay revealed that, compared with the spinal cord injury group, GPX4, xCT, and glutathione expression was markedly increased in the Deferoxamine group.(5) Real-time polymerase chain reaction revealed that, compared with the Deferoxamine group, mRNA levels of ferroptosis-related genes Acyl-CoA synthetase family member 2(ACSF2) and iron-responsive element-binding protein 2(IREB2) were up-regulated in the Deferoxamine group.(6) Deferoxamine increased survival of neurons and inhibited gliosis. These findings confirm that Deferoxamine can repair spinal cord injury by inhibiting ferroptosis. Targeting ferroptosis is therefore a promising therapeutic approach for spinal cord injury. 相似文献
80.
目的研究快速成型(RP)技术辅助下制作的个体化假体复合珊瑚羟基磷灰石(CHA)、重组人骨形成蛋白2(rhBMP-2)修复兔下颌骨缺损的成骨效果。
方法以27只新西兰大白兔为实验对象,随机数字表法平均分成3组(每组9只),全部建立下颌骨连续性缺损模型,并在兔下颌骨缺损区分别植入个体化假体+自体骨(A组)、个体化假体+CHA(B组)、个体化假体+CHA+rhBMP-2(C组)。分别于术后4、12、24周3个时间点处死动物取材,进行大体标本观察,以及骨钙素(OC)、Ⅰ型胶原(COL-1)的免疫组化观察,分别比较各组修复骨缺损的能力,并对实验数据进行重复测量设计资料的单因素方差分析。
结果术后24周各组实验兔外形均对称,通过OC及COL-1的吸光度检测,骨缺损区均有大量新骨形成,A组(0.537 ± 0.010)、C组(0.530 ± 0.010)可见大量骨小梁及编织骨结构,缺损区的新骨OC、COL-1的免疫组化观察基本一致,差异无统计学意义(t = 0.007,P>0.05);但A组强于B组(0.415 ± 0.009,t = 0.122,P<0.001);C组也强于B组(t = 0.121,P<0.001),差异均有统计学意义。
结论在兔下颌骨缺损修复中,通过RP技术和组织工程技术相结合,CHA复合rhBMP-2后成骨能力明显增强,成骨效能肯定,为后期的临床应用提供可靠的实验基础。 相似文献