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1.
Paraneoplastic syndromes consist on systemic manifestations associated with certain cancers which are not a direct consequence of tumor invasion or its metastases. It is known that autoimmunity and autoantibody synthesis play an important role in its pathophysiology due to a process of molecular mimicry. Paraneoplastic syndromes in ophthalmology are rare, but it is important to recognize them clinically because in some cases symptoms preceded the diagnosis of an underlying neoplasia. Most frequently involved cancer is small cell lung carcinoma, but there is also a relationship with other tumor etiologies such as thymoma, gynecological tumors or neuroblastoma in children. Paraneoplastic syndromes with ocular involvement can be divided into those that affect the afferent visual pathway, such as cancer-associated retinopathy, melanoma-associated retinopathy, or paraneoplastic optic neuropathy; and the ones that affect the efferent visual pathway, such as bilateral tonic pupils, Myasthenia Gravis, Lambert-Eaton syndrome or paraneoplastic cerebellar degeneration. The presence of autoantibodies is helpful in clinical practice but negativity does not exclude this diagnosis. Although evolution and prognosis is linked to primary disease, in some cases specific treatment, usually immunosuppressive therapy, can help improving patients quality of life.  相似文献   
2.
目的 观察重组人乙酰胆碱受体(H-AChR) γ亚单位免疫HLA-DQ8转基因小鼠眼肌型实验性自身免疫性重症肌无力(oEAMG)模型体外培养上清液中Th1/Th2细胞因子水平变化,探讨其与oEAMG免疫学发病机制的联系。方法 HLA-DQ8转基因小鼠用乳化于完全弗氏佐剂(CFA)的重组H-AChR γ亚单位,或E. Coli提取物,或纯CFA分别免疫,分别于第0天、第30天和第60天共免疫3次。第3次免疫后28 d,处死小鼠后取淋巴结和脾淋巴细胞体外培养,收集培养上清液,采用ELISA法检测白细胞介素(IL)-2、γ-干扰素(IFN-γ)、IL-6和IL-10水平。结果 H-AChR γ组小鼠淋巴结及脾淋巴细胞体外培养上清液中IL-2(F=42.835,P<0.001;F=38.030,P<0.001)和INF-γ水平(F=76.332,P<0.001;F=34.865,P<0.001)均明显高于E. coli组和CFA免疫组;IL-6(F=1.325,P=0.284;F=1.935,P=0.166)和IL-10水平(F=0.908,P=0.417;F=1.189,P=0.322)与E. coli 组和CFA组差异无统计学意义。结论 Th1细胞因子在重组H-AChR γ亚单位诱导HLA-DQ8转基因小鼠所建立oEAMG模型的发病机制中可能发挥重要作用,而Th2细胞及其细胞因子作用尚不十分明确。  相似文献   
3.
目的探讨重症肌无力(myasthenia gravis,MG)患者的认知功能特征。方法纳入83例MG患者和39例健康对照者,应用简易智能状态检查量表(mini-mental status examination,MMSE)、加利福尼亚语言学习测验(California verbal learning test,CVLT)、简易视觉空间记忆测验(brief visuospatial memory test-revised,BVMT-R)、符号数字模式测验(symbol digit modalities test,SDMT)、Benton线方向判断测验(benton judgment of line orientation test,BJLOT)、定步调听觉连续加法测验(paced auditory serial addition test,PASAT)、言语流畅性测验(verbal fluency test,VFT)进行认知功能评估,应用贝克抑郁测验(Beck depression inventory,BDI)评估抑郁状态,对两组资料进行对比分析,并进一步评估疾病分型、合并症情况、病程、疾病严重程度、药物治疗情况等临床特征对MG组认知损害的影响。结果MG组MMSE[28(26,29)分,29(28,30)分]、CVLT、BVMT-R、SDMT[(37.06±12.18)分,(47.54±14.91)分]、PASAT[(32.86±10.23)分,(37.00±8.82)分]评分低于对照组,差异具有统计学意义(P<0.05),两组间BJLOT、VFT、BDI评分差异无统计学意义(均P>0.05)。Spearman相关分析显示重症肌无力患者疾病严重程度定量评分(quantitative MG score,QMG)与SDMT得分存在负相关(r=-0.234,P<0.05);MG日常生活质量量表(MG-activities of daily living profile,MG-ADL)评分与BVMT-R 3试总和得分存在负相关(r=-0.283,P<0.05)。结论重症肌无力存在认知功能损害,主要表现在记忆力、注意力、信息处理速度、视觉记忆,提示重症肌无力可能存在中枢神经系统受累。部分认知领域的损害可能与疾病严重程度相关。临床医生在工作中应加强对MG患者认知障碍的认识,早期评估,密切随访,适当干预。  相似文献   
4.

Objective

To investigate the efficacy and safety of an influenza vaccination in patients with myasthenia gravis with acetylcholine receptor antibodies (AChR MG).

Methods

An influenza vaccination or placebo was administered to 47 AChR MG patients. Before and 4?weeks after administration blood samples and clinical outcome scores were obtained. Antibodies to the vaccine strains A/California/7/2009 (H1N1)pdm09, A/Hong Kong/4801/14 (H3N2) and B/Brisbane/060/08 were measured using the hemagglutination-inhibition (HI) assay and disease-specific AChR antibody titers were measured with a radio-immunoprecipitation assay. Forty-seven healthy controls (HC) were vaccinated with the same influenza vaccine to compare antibody titers.

Results

A post-vaccination, seroprotective titer (HI?≥?1:40) was achieved in 89.4% of MG patients vs. 93.6% in healthy controls for the H3N2 strain, 95.7% vs 97.9% for the H1N1 strain and 46.8 vs 51% for the B-strain. A seroprotective titer for all three strains of the seasonal influenza vaccine was reached in 40.4% (19/47) of the MG group and in 51% (24/47) of the HC group. Immunosuppressive medication did not significantly influence post geomean titers (GMT). The titers of disease-specific AChR antibodies were unchanged 4?weeks after vaccination. The clinical outcome scores showed no exacerbation of MG symptoms.

Conclusion

The antibody response to an influenza vaccination in patients with AChR MG was not different from that in healthy subjects, even in AChR MG patients using immunosuppressive medication. Influenza vaccination does not induce an immunological or clinical exacerbation of AChR MG.

Clinical trial registry

The influenza trial is listed on clinicaltrialsregister.eu under 2016-003138-26.  相似文献   
5.
周婷婷  张艺  樊展  胡晔  武彩花 《陕西中医》2020,(11):1665-1668
目的:探讨补脾益肾方联合温针灸治疗对重症肌无力(MG)疗效及对免疫功能的影响。方法:随机分配84例MG病例为西药组和针药组,每组各42例,西药组给予常规西药治疗,即泼尼松片中剂量冲击,小剂量隔日维持治疗,针药组基于以上用药基础给予补脾益肾方联合温针灸治疗,治疗3个月后,统计两组治疗前后的中医证候积分,评估两组中医证候疗效,对比治疗前后的颈部血管流速、T淋巴细胞亚群水平和血清可溶性白细胞介素6受体水平。结果:治疗后,两组中医证候积分显著降低,针药组的变化幅度大于西药组(P<0.05); 针药组的中医证候总有效率低于西药组(P<0.05); 治疗后,两组颈内动脉(ICA)、颈总动脉(CCA)、颈外动脉(ECA)显著提高(P<0.05),两组T淋巴细胞中CD3+、CD4+亚群所占比和CD8+、CD4+比值显著降低(P<0.05),两组血清slL-6R水平均显著降低(P<0.05),以上指标针药组变化幅度大于西药组(P<0.05)。结论:补脾益肾方联合温针灸治疗能缓解MG患者的中医证候症状,提高疗效,促进其颈部血管循环,纠正患者自身机体免疫功能紊乱。  相似文献   
6.
7.
8.
[目的] 总结分析裘昌林教授救治重症肌无力危象的中医辨治思路。[方法] 通过跟师学习、整理医案、复习相关中医文献,整理总结裘师临床抢救重症肌无力危象的中医辨治经验,分析重症肌无力危象的中医认识,中医治疗原则和用药方法,并列举医案分析。[结果] 裘师认为,重症肌无力危象病情危重,属于中医大气下陷证,与脾肾密切相关,脾肾虚损为本,甚者脉微息弱、元气耗散而气脱,治疗重在急救脱陷、温肾敛阴,兼顾祛痰通腑,并且研用马钱子起沉疴。所举医案贯彻裘师辨治经验,遣方用药以健脾益气为主,补益肝肾为辅,佐以祛痰通腑,获得较好疗效。[结论] 裘师基于中医病因病机,对重症肌无力危象进行辨证论治,遣方用药从虚出发,随证加减,配合西医治疗,能够提高疗效,减少不良反应,充分体现了中医药治疗重症肌无力危象的优势,其经验具有推广价值。  相似文献   
9.
Although quantitative MRI can be instrumental in the diagnosis and assessment of disease progression in orbital diseases involving the extra‐ocular muscles (EOM), acquisition can be challenging as EOM are small and prone to eye‐motion artefacts. We explored the feasibility of assessing fat fractions (FF), muscle volumes and water T2 (T2water) of EOM in healthy controls (HC), myasthenia gravis (MG) and Graves' orbitopathy (GO) patients. FF, EOM volumes and T2water values were determined in 12 HC (aged 22‐65 years), 11 MG (aged 28‐71 years) and six GO (aged 28‐64 years) patients at 7 T using Dixon and multi‐echo spin‐echo sequences. The EOM were semi‐automatically 3D‐segmented by two independent observers. MANOVA and t‐tests were used to assess differences in FF, T2water and volume of EOM between groups (P < .05). Bland–Altman limits of agreement (LoA) were used to assess the reproducibility of segmentations and Dixon scans. The scans were well tolerated by all subjects. The bias in FF between the repeated Dixon scans was ?0.7% (LoA: ±2.1%) for the different observers; the bias in FF was ?0.3% (LoA: ±2.8%) and 0.03 cm3 (LoA: ± 0.36 cm3) for volume. Mean FF of EOM in MG (14.1% ± 1.6%) was higher than in HC (10.4% ± 2.5%). Mean muscle volume was higher in both GO (1.2 ± 0.4 cm3) and MG (0.8 ± 0.2 cm3) compared with HC (0.6 ± 0.2 cm3). The average T2water for all EOM was 24.6 ± 4.0 ms for HC, 24.0 ± 4.7 ms for MG patients and 27.4 ± 4.2 ms for the GO patient. Quantitative MRI at 7 T is feasible for measuring FF and muscle volumes of EOM in HC, MG and GO patients. The measured T2water was on average comparable with skeletal muscle, although with higher variation between subjects. The increased FF in the EOM in MG patients suggests that EOM involvement in MG is accompanied by fat replacement. The unexpected EOM volume increase in MG may provide novel insights into underlying pathophysiological processes.  相似文献   
10.
Background and PurposeA major concern with ocular myasthenia gravis (MG) is the potential conversion to generalized MG. This study was conducted to determine if the repetitive nerve stimulation (RNS) test could predict the conversion from ocular to generalized MG.MethodsThe RNS test was conducted in a consistent manner on five muscles in the face and limbs in every patient. Subjects were divided into those who remained as ocular MG (ROMG group) and those who experienced conversion to generalized MG during follow-up (GOMG group).ResultsConversion to generalized MG occurred in 24 (21.4%) of 112 MG patients with ocular onset. The proportion of patients displaying abnormal decreases in responses in the trapezius, abductor digiti minimi, or flexor carpi ulnaris muscles on the RNS test was higher in the GOMG group (p<0.001, p=0.002, and p<0.001, respectively). The Cox proportional-hazards model revealed that an abnormal result on the RNS test was significantly associated with conversion to generalized MG [hazard ratio (HR)=3.13, 95% confidence interval (CI)=1.18–8.32]. Notably, the HR was higher for abnormal results on the RNS test for the limb muscles, at 5.19 (95% CI=2.09–12.90).ConclusionsAn abnormal result on the RNS test, especially in the limb muscles, is an independent predictor of the conversion from ocular to generalized MG. Applying the RNS test to limb muscles could be useful for predicting the conversion to generalized MG in patients with ocular onset.  相似文献   
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