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Fabry肾病临床病理观察 总被引:2,自引:0,他引:2
目的阐明具有肾损害的Fabry病的临床病理特点。方法回顾性分析2例具有肾损害的Fabry病患者的临床表现,并对其肾活检组织进行光镜、免疫荧光及超微结构观察。结果2例患者出现蛋白尿、慢性肾功能不全/慢性肾炎;光镜下可见肾小球脏层上皮细胞空泡变性,远端肾小管上皮细胞、肾小球内皮细胞及系膜细胞、间质成分也可出现泡沫样变;病变晚期,肾小球出现节段性和/或球性硬化;超微结构显示肾小球脏层上皮细胞内具有嗜锇性髓鞘样包涵体,表现为“斑马”样外观。结论Fabry病少见。具有肾损害的患者临床可出现蛋白尿,晚期常发展为肾功能衰竭,肾小球脏层上皮细胞内出现嗜锇性髓鞘样小体是Fabry病特征性的形态学改变。 相似文献
34.
对于大量脑室内出血铸型的患者,我院以往多采用双侧侧脑室穿刺引流术治疗,由于不能快速缓解颅内高压而疗效不理想。于2006年我院开始应用神经内镜的微创手术方法治疗2例脑室内出血铸型,均取得良好效果,现报告如下。 相似文献
35.
正慢性肾脏病(CKD)现已成为全球性公共健康问题,其在我国的患病率为10. 8%[1]。在CKD患者中,几乎都存在着不同程度的钙磷代谢紊乱,长期的钙磷代谢紊乱可引发甲状旁腺功能亢进、矿物质和骨代谢异常、心血管事件等,严重影响着CKD的进展和预后。过去一般认为活性维生素D、甲状旁腺激素(PTH)是钙磷代谢中最重要的调节因子,近年来随着FGF23的发现,又为该领域提供了新的视野。FGF23与1,25(OH)2VitD3、PTH共同组成骨-肾-甲状旁腺内分泌轴,在钙磷调节中起着 相似文献
36.
Objective To investigate the prevalence and risk factors of chronic kidney disease (CKD) in the general adult population in the Hulunbeir Prefecture, Inner Mongolia Autonomous Region where many minorities of north China live. Methods Sampling surveywas performed in the residents aged 20 years and older in the Hulunbeir Prefecture. All the investigated subjects were tested for urinary albumin to creatinine ratio (ACR); hematuria by microscopy of urinary sediment; and GFR estimated by modified MDRD equation for Chinese adults (eGFR). The related risk factors of CKD were also investigated. Results A total of 4522 subjects were enrolled in the study. The prevalence of albuminuria was 7.11%, hematuria was 2.64% and reduced eGFR [60 ml-min-1·(1.73 m2)-1] was 2.75%. The prevalence of hypertension was 38.90%; hyperglycemia 6.61%; hyperlipidemia 2.72%; increased waist 24.79% and metabolic syndrome 15.02%. After the subjects with combined microalbuminuria, hematuria and reduced eGFR were excluded, the prevalence of CKD was 12.95%. Logistic regression analysis and stratified analysis showed increased age, increased waist, elevated systolic pressure, hyperglycemia,hypertriglyceridemia and metabolic syndrome were independently associated with albuminuria;increased age, elevated systolic pressure and hyperglycemia were independently associated with reduced eGFR; increased age was independently associated with hematuria. Conclusions The prevalence of adult CKD is 12.95% in the Hulunbeir Prefecture, Inner Mongolia Autonomous Region. Independent risk factors of CKD include increased age, increased waist, hypertension,abnormal blood glucose or lipid, and metabolic syndrome. 相似文献
37.
目的 确定国人肾小球大小正常值并探讨肾小球直径测量法。 方法 选择我科1998至2008年肾穿刺病理检查病例中体质量及血糖正常、病理诊断为肾小球轻微病变或薄基底膜肾病的100例患者作为正常值检测组,在其肾小球最大剖面上用直接及间接两种方法测量毛细血管袢直径。同时随意选择体质量及血糖正常的微小病变肾病、局灶节段性肾小球硬化(FSGS)及膜性肾病患者各10例,进行上述肾小球直径测量,并与正常值检测结果比较。 结果 直接和间接测量法结果一致,性别与年龄对肾小球直径无明显影响。通过不同方法获得的肾小球毛细血管袢直径正常值范围如下:(1)含极(血管极、尿极)肾小球:直接法结果为101.3~182.9 μm;间接法为100.3~181.5 μm;(2)含极肾小球加大于它们的无极肾小球:直接法结果为108.5~182.9 μm;间接法为107.6~183.2 μm。另外,30例肾脏病患者的肾小球直径均在此正常值范围内。 结论 本研究应用的两种肾小球直径检测法均可行,获得的国人肾小球直径正常值范围可以推广。 相似文献
38.
目的 探讨我国内蒙古自治区呼伦贝尔少数民族聚居区成年人群中慢性肾脏病(CKD)患病率及其危险因素。 方法 对该地区20岁以上常住居民进行CKD抽样调查,被调查者均检测了尿白蛋白/肌酐比率、血尿(离心后尿沉渣显微镜检查)及估计肾小球滤过率(eGFR,检验血清肌酐后用国人校正的简化MDRD公式计算);并同时调查了CKD的相关危险因素。 结果 符合入选条件的被调查者共4522例,白蛋白尿阳性率为7.11%;血尿阳性率为2.64%;eGFR低于60 ml8226;min-18226;(1.73 m2)-1者为2.75%;去除白蛋白尿、血尿及eGFR下降共同存在造成的重复,该地区CKD患病率为12.95%。高血压患病率38.90%,糖代谢异常6.61%,脂代谢异常34.60%,腰围增大24.79%,代谢综合征15.02%。多因素Logistic回归分析及分层分析显示,年龄增加、腰围增大、收缩压升高、空腹血糖升高、血清三酰甘油增高及患代谢综合征与白蛋白尿发生相关;年龄增加、收缩压升高及空腹血糖升高与肾功能下降相关;年龄增加与血尿发生相关。 结论 内蒙古自治区呼伦贝尔地区CKD患病率为12.95%。相关危险因素包括年龄增加、腰围增大、高血压、血糖或血脂异常、及代谢综合征。 相似文献
39.
目的:合成木犀草素Mannich碱衍生物并考察其抗癌活性。方法:室温下木犀草素与甲醛、胺经Mannich反应得到8种Mannich碱衍生物。采用MTT法,以5氟-尿嘧啶(5-Fu)为阳性对照药,通过人宫颈癌细胞(Hela)、人胃癌细胞(SGC-7901)、人结肠癌细胞(HCT-116)、人白血病细胞(K562)、人乳腺癌细胞(MCF-7)、人前列腺癌细胞(DU-145)等6种肿瘤细胞进行体外抗癌活性评价,以正常人胚肾上皮细胞(HEK-293)为毒性对照;对化合物8h进行抗癌分子机制研究。结果:合成的8种化合物结构经1H-NMR、13C-NMR和MS确证。体外抗癌活性试验表明部分化合物显示出比木犀草素更好的抗癌活性。结论:化合物8h可能通过线粒体途径抑制SGC-7901细胞增殖,从而诱导细胞凋亡。 相似文献
40.
内蒙古呼伦贝尔地区成人慢性肾脏病流行病学调查 总被引:3,自引:0,他引:3
Objective To investigate the prevalence and risk factors of chronic kidney disease (CKD) in the general adult population in the Hulunbeir Prefecture, Inner Mongolia Autonomous Region where many minorities of north China live. Methods Sampling surveywas performed in the residents aged 20 years and older in the Hulunbeir Prefecture. All the investigated subjects were tested for urinary albumin to creatinine ratio (ACR); hematuria by microscopy of urinary sediment; and GFR estimated by modified MDRD equation for Chinese adults (eGFR). The related risk factors of CKD were also investigated. Results A total of 4522 subjects were enrolled in the study. The prevalence of albuminuria was 7.11%, hematuria was 2.64% and reduced eGFR [60 ml-min-1·(1.73 m2)-1] was 2.75%. The prevalence of hypertension was 38.90%; hyperglycemia 6.61%; hyperlipidemia 2.72%; increased waist 24.79% and metabolic syndrome 15.02%. After the subjects with combined microalbuminuria, hematuria and reduced eGFR were excluded, the prevalence of CKD was 12.95%. Logistic regression analysis and stratified analysis showed increased age, increased waist, elevated systolic pressure, hyperglycemia,hypertriglyceridemia and metabolic syndrome were independently associated with albuminuria;increased age, elevated systolic pressure and hyperglycemia were independently associated with reduced eGFR; increased age was independently associated with hematuria. Conclusions The prevalence of adult CKD is 12.95% in the Hulunbeir Prefecture, Inner Mongolia Autonomous Region. Independent risk factors of CKD include increased age, increased waist, hypertension,abnormal blood glucose or lipid, and metabolic syndrome. 相似文献