野生型胃肠道间质瘤的研究进展

胃肠道间质瘤(GIST)是最常见的消化道间叶组织来源肿瘤,约10%的GIST患者分子检测无KIT/PDGFRA基因突变,称为野生型GIST。根据是否有琥珀酸脱氢酶B(SDHB)表达缺失,野生型GIST可分为SDH缺陷型和非SDH缺陷型,SDH缺陷型包括无综合征相关性、Carney三联征相关性及Carney-stratakis综合征相关性GIST;非SDH缺陷型包括BRAF突变、Ⅰ型神经纤维瘤病相关性、K/N-RAS突变及四重野生型GIST等。野生型GIST的发生发展、临床病理特征和治疗原则均与KIT或PDGFRA突变的GIST有较大差异。本文就野生型GIST的分子机制和临床诊疗进展做一综述。     

持续性特发性耳鸣严重程度的相关因素分析

目的 探讨导致持续性特发性耳鸣严重程度的相关因素。 方法 分析870例持续性特发性主观性耳鸣患者的一般资料、病史、听力学结果、耳鸣匹配实验、耳鸣残疾量表、匹兹堡睡眠质量指数量表和焦虑自评量表,应用多因素Logistic回归分析研究与患者耳鸣严重程度相关的因素。 结果 (1)性别(P<0.001)、年龄(P=0.010)、耳鸣主调声频率(P=0.005)、听力损失(P=0.037)、焦虑程度(P<0.001)、睡眠状况(P<0.001)在THI分级上差异有统计学意义(P<0.05);(2)病程(P=0.053)、侧别(P=0.437)、主调声响度(P=0.120)在THI分级上差异均无统计学意义(P>0.05)。 结论 女性患者特发性耳鸣的程度较男性更为严重;低频较高频更扰人;焦虑程度和睡眠状况是影响耳鸣严重程度的因素,而病程、侧别、耳鸣主调声响度不是影响患者特发性耳鸣严重程度的因素。     

成人Still病诊疗进展

【摘要】 成人Still病是一类病因未明的全身炎症性疾病，临床表现多样，其诊断和治疗存在一定困难。近年来发现，血红素加氧酶1、钙网蛋白、炎症细胞因子、糖基化终末产物等新型标志物可对成人Still病的活动程度和严重程度进行全面评估，而新研发的生物制剂如肿瘤坏死因子抑制剂、白细胞介素1（IL-1）抑制剂、IL-6抑制剂、重组IL-18结合蛋白也有望用于治疗。本文主要综述成人Still病的诊断和治疗进展。     

Comparison of Salivary and Serum Alkaline Phosphates Level and Lactate Dehydrogenase Levels in Patients with Tobacco Related Oral Lesions with Healthy Subjects - A Step Towards Early Diagnosis

Aim: To evaluate and compare salivary and serum levels of Alkaline Phosphates and Lactate Dehydrogenase in patients without the habit of tobacco, in patients with the habit of tobacco, in patients with benign oral lesions and in patients with oral premalignant lesions and oral malignant lesions. Material and Methodology: This study was comprised of 500 subjects, Group I: 100 healthy individuals without the habit of tobacco usage formed the control group. Group II: 100 patients with the habit of tobacco/ smoking consumption without any oral lesion. Group III: 100 patients with benign oral lesions. Group IV: 100 patients having the history of tobacco consumption and having apparent precancerous lesions like leukoplakia, erythroplakia. Group V:100  patients having frank oral cancer. The grade of dysplasia in these patients was statically correlated with the levels of serum and salivary ALP and LDH. Results: This study revealed that there was high expression of both serum and salivary ALP and LDH  in group IV and Group V as compared with the other groups and mean difference showed a statistically significant p value of less than 0.01. This study revealed that the in group V, the highest level of serum and salivary ALP was found in those patients who were reported with poorly differentiated oral cancer. Conclusion: Both Alkaline phosphates and Lactate dehydrogenase could be considered a sensitive markers for the detection of dysplasia with already existing precancancerous and cancerous lesions.     

外泌体在血液肿瘤微环境中的作用及其诊治意义

外泌体是细胞外囊泡的一类主要成分，是正常生理情况下组织细胞间信号传递的重要介质；在多种血液肿瘤研究中发现外泌体是骨髓微环境各成分间相互作用的关键载体，由于其广泛存在并含有特定的核酸、蛋白等分子，外泌体被作为潜在的生物标志物和治疗靶点/载体，在血液肿瘤诊治中的重要性近年正逐步受到关注。     

Influence of respiratory allergy and restless sleep on definite sleep bruxism: a cross-sectional clinical study

ObjectiveThis cross-sectional clinical study evaluated the associations between sociodemographic, occupational, clinical conditions, psychological and sleep quality variables on definite sleep bruxism (SB).MethodsAll records obtained from adults (aged 20–60 years) and the elderly (aged >60 years) who had undergone polysomnography (PSG) at a private medical outpatients’ clinic from July 2017 to February 2018 were reviewed. Data from a questionnaire, based on the criteria of the American Academy of Sleep Medicine (AASM), were also gathered. Definite SB data pattern distribution was analyzed, and multivariate Poisson regression with robust variance was used to assess the associations between definite SB diagnosis, determined via PSG recordings, and the independent variables. A significance level of 5% was adopted.ResultsA total of 240 individuals were included in the study and the SB prevalence was 7.08% (n = 17). The adjusted Poisson regression analysis revealed association between definitive SB and individuals with respiratory allergy (PR = 3.63; 95% CI:1.01–13; P = 0.047) and restless sleep (PR = 2.97; 95% CI:1.04–8.50; P = 0.042).ConclusionThis study found associations between definite SB and clinical conditions (respiratory allergy) and sleep behavior (restless sleep). Knowledge regarding factors associated with definite SB can contribute to decision making in the clinical setting and management strategies involving a multidisciplinary approach.     

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目的对儿童血管迷走性晕厥(VVS)的传统诊断程序与新诊断程序进行卫生经济学评价。方法采用回顾分析方法,根据不同的诊断程序(即传统诊断程序和新诊断程序),将1995年1月至2005年5月北京大学第一医院儿科确诊的81例VVS患儿分为2组,分别计算其就诊费用、检查费用、住院日、确诊日等,运用经济学最小成本分析法进行评价。结果新诊断程序的平均就诊费用比传统诊断程序少耗费1668.80元;新诊断程序的平均就诊检查费用比传统诊断程序少耗费1413.30元。结论新诊断程序对确诊儿童血管迷走性晕厥具有良好的经济学效益,值得临床推广。     

腹部恶性纤维组织细胞瘤28例临床分析

目的探讨腹部恶性纤维组织细胞瘤的诊治方法。方法回顾性分析28例腹部恶性纤维组织细胞瘤的临床表现、影像学特征、病理学特点、治疗方法、疗效和预后。结果28例中17例来源于腹膜后，6例来源于腹腔脏器，3例来源于肠系膜，2例来源于腹壁，主要临床表现为明显消瘦，体重减轻、腹痛和腹部肿块。B超和CT扫描提示腹部占位性病变。肿瘤≥2个24例，单发4例。根治性切除17例，部分切除11例，术后联合化疗16例，联合放疗14例。术后1、3、5年生存率分别为76．9％，26．9％和3．9％。结论腹部恶性纤维组织细胞瘤主要来源于腹膜后，常多发，手术切除是首选的治疗方法，术后联合放化疗，可能改善患者预后。