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Background/PurposeA small number of Hirschsprung disease (HD) patients develop inflammatory bowel disease (IBD)-like symptoms after pullthrough surgery. The etiology and pathophysiology of Hirschsprung-associated IBD (HD-IBD) remains unknown. This study aims to further characterize HD-IBD, to identify potential risk factors and to evaluate response to treatment in a large group of patients.MethodsRetrospective study of patients diagnosed with IBD after pullthrough surgery between 2000 and 2021 at 17 institutions. Data regarding clinical presentation and course of HD and IBD were reviewed. Effectiveness of medical therapy for IBD was recorded using a Likert scale.ResultsThere were 55 patients (78% male). 50% (n = 28) had long segment disease. Hirschsprung-associated enterocolitis (HAEC) was reported in 68% (n = 36). Ten patients (18%) had Trisomy 21. IBD was diagnosed after age 5 in 63% (n = 34). IBD presentation consisted of colonic or small bowel inflammation resembling IBD in 69% (n = 38), unexplained or persistent fistula in 18% (n = 10) and unexplained HAEC >5 years old or unresponsive to standard treatment in 13% (n = 7). Biological agents were the most effective (80%) medications. A third of patients required a surgical procedure for IBD.ConclusionMore than half of the patients were diagnosed with HD-IBD after 5 years old. Long segment disease, HAEC after pull through operation and trisomy 21 may represent risk factors for this condition. Investigation for possible IBD should be considered in children with unexplained fistulae, HAEC beyond the age of 5 or unresponsive to standard therapy, and symptoms suggestive of IBD. Biological agents were the most effective medical treatment.Level of EvidenceLevel 4  相似文献   
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目的 探讨血浆外泌体源性miR-1224-5p与溃疡性结肠炎(ulcerative colitis,UC)患者炎性反应状态及预后的关系。方法 选择2018年1月-2019年10月海安市人民医院收治的103例UC患者,其中活动期57例,缓解期46例;选择同期来我院体检的60例健康人群作为对照组。采用实时荧光定量聚合酶链反应检测血浆外泌体源性miR-1224-5p表达量。比较各组血浆外泌体源性miR-1224-5p表达差异,采用Pearson相关分析探讨血浆外泌体源性miR-1224-5p与Mayo评分、Baron评分的相关性,多因素Logistic回归分析影响UC患者预后复发的相关因素。结果 活动期UC患者血浆外泌体源性miR-1224-5p明显高于缓解期患者与对照组,缓解期UC患者血浆外泌体源性miR-1224-5p明显高于对照组(P<0.05)。活动期重度组UC患者血浆外泌体源性miR-1224-5p、Mayo评分、Baron评分均明显高于中度组和轻度组,中度组UC患者血浆外泌体源性miR-1224-5p、Mayo评分、Baron评分均明显高于轻度组(P<0.05)。Pearson相关分析发现,血浆外泌体源性miR-1224-5p与Mayo评分、Baron评分均呈正相关(P<0.05)。活动期UC患者复发率(38.60%,22/57)明显高于缓解期UC患者(19.56%,9/46)(χ2=4.382,P=0.036)。复发组UC患者血浆外泌体源性miR-1224-5p及Mayo评分、Baron评分均明显高于未复发组(P<0.05)。多因素Logistic回归分析显示,血浆外泌体源性miR-1224-5p、Mayo评分、Baron评分、疾病处于活动期均为影响UC复发的危险因素(P<0.05)。结论 溃疡性结肠炎患者血浆外泌体源性miR-1224-5p相对表达量明显升高,活动期UC患者血浆外泌体源性miR-1224-5更高,且与患者炎性反应程度呈正相关,同时是UC患者复发的相关因素。  相似文献   
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Cutaneous tuberculosis classically presents as Lupus vulgaris, scrofuloderma, tuberculosis verrucose cutis and tubercular abscess. Hypersensitivity reaction to the bacilli leads to Lichen scrofulosorum and papulonecrotic tuberculids. At the same time, it can have myriad of clinical presentations, many of which are still undescribed. It is important to regularly update ourselves with these unusual manifestations so as to ensure early treatment and reduction of overall morbidity. In this case series tuberculosis manifesting as rapidly progressing diffuse facial granulomas, sporotrichoid tuberculosis, tuberculosis mimicking squamous cell carcinoma, scrofuloderma as tubercular ulcer, lupus vulgaris with nasal septal perforation, lupus vulgaris resembling furuncle, psoriasis, dermatitis and BT Hansen are described in immunocompetent individuals. These cases highlight the importance of recognition of atypical forms of cutaneous tuberculosis to minimize scarring and dissemination of bacilli.  相似文献   
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目的:探讨葛根芩连汤上调微RNA-542-3p(miR-542-3p)表达保护溃疡性结肠炎(UC)大鼠的机制研究。方法:选取8~10周龄SD大鼠经UC造模及鉴定后分成模型组、美沙拉嗪组、葛根芩连汤低剂量、中剂量、高剂量组,同批次等体质量健康大鼠作为空白对照组,每组16只。UC结肠细胞进入对数生长期后消化传代至24孔板培养,每孔加入2×105个细胞,用Lipofectamine 3000转染miR-NC、miR-542-3p、anti-miR-NC、anti-miR-542-3p质粒至UC大鼠结肠上皮细胞并分组,miR-NC、miR-542-3p组细胞经含10%生理盐水处理24 h,而anti-miR-NC、anti-miR-542-3p组用含10%葛根芩连汤处理24 h,处理结束后分析细胞上清液炎症介质和氧化应激相关指标的变化。酶联免疫吸附试验法(ELISA)检测大鼠结肠组织和细胞肿瘤坏死因子-α(TNF-α)、白细胞介素-6(IL-6)、白细胞介素-8(IL-8)含量;化学比色法检测丙二醛(MDA)、超氧化物歧化酶(SOD)、谷胱甘肽过氧化物酶(GSH-Px)含量;实时荧光定量聚合酶链式反应(RT-qPCR)检测miR-542-3p表达水平。结果:与模型组比较,葛根芩连汤中剂量、高剂量组TNF-α、IL-6、IL-8含量显著降低(P<0.05)。与模型组比较,葛根芩连汤中剂量、高剂量组MDA水平显著降低,SOD、GSH-PX水平显著升高(P<0.05)。与模型组比较,葛根芩连汤中、高剂量组miR-542-3p表达水平显著升高(P<0.05)。与UC+miR-NC组比较,UC+miR-542-3p组细胞miR-542-3p表达水平显著升高;TNF-α、IL-6、IL-8含量显著降低;MDA水平显著降低以及SOD、GSH-Px水平显著升高(P<0.05)。与UC+anti-miR-NC+葛根芩连汤组比较,UC+anti-miR-542-3p+葛根芩连汤组细胞miR-542-3p表达水平显著降低;TNF-α、IL-6、IL-8含量显著升高;MDA水平显著升高以及SOD、GSH-Px水平显著降低(P<0.05)。结论:葛根芩连汤上调miR-542-3p表达保护UC大鼠,消除肠道炎症。  相似文献   
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Inflammatory bowel disease, encompassing Crohn’s disease (CD) and ulcerative colitis, are chronic immune-mediated inflammatory bowel diseases (IBD) that primarily affect the gastrointestinal tract with periods of activity and remission. Large body of evidence exist to strengthen the prognostic role of endoscopic evaluation for both disease activity and severity and it remains the gold standard for the assessment of mucosal healing. Mucosal healing has been associated with improved clinical outcomes with prolonged remission, decreased hospitalization, IBD-related surgeries and colorectal cancer risk. Therefore, endoscopic objectives in IBD have been incorporated as part of standard care. With the known increased risk of colorectal cancer in IBD, although prevention strategies continue to develop, regular surveillance for early detection of neoplasia continue to be paramount in IBD patients’ care. It is thanks to evolving technology and visualization techniques that surveillance strategies are continuously advancing. Therapeutic endoscopic options in IBD have also been expanding, from surgery sparing therapies such as balloon dilation of fibrostenotic strictures in CD to endoscopic mucosal resection of neoplastic lesions. In this review article, we discuss the current evidence on the use of endoscopy as part of standard of care of IBD, its role in surveillance of neoplasia, and the role of interventional endoscopic therapies.  相似文献   
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Inflammatory bowel disease (IBD) is a chronic disease that requires chronic treatment throughout the evolution of the disease, with a complex physiopathology that entails great challenges for the development of new and specific treatments for ulcerative colitis and Crohn´s disease. The anti-tumor necrosis factor alpha therapy has impacted the clinical course of IBD in those patients who do not respond to conventional treatment, so there is a need to develop new therapies and markers of treatment response. Various pathways involved in the development of the disease are known and the new therapies have focused on blocking the inflammatory process at the gastrointestinal level by oral, intravenous, subcutaneous, and topical route. All these new therapies can lead to more personalized treatments with higher success rates and fewer relapses. These treatments have not only focused on clinical remission, but also on achieving macroscopic changes at the endoscopic level and microscopic changes by achieving mucosal healing. These treatments are mainly based on modifying signaling pathways, by blocking receptors or ligands, reducing cell migration and maintaining the integrity of the epithelial barrier. Therefore, this review presents the efficacy and safety of the new treatments that are currently under study and the advances that have been made in this area in recent years.  相似文献   
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