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1.
??Abstract?? Systemic vasculitis is a group of vasculitis with common inflammation of the blood vessel and involves multiple organs. It can involve all kinds of vessels of the organs??showing different clinical manifestations and lacking specificity. Through summarizing the clinical manifestation and diagnosis of some common systemic vasculitis in children??we aim to improve pediatricians’s awareness of these diseases??in order to early discover??diagnose and improve the prognosis.  相似文献   
2.
??Objective To observe the changes of red cell distribution width ??RDW?? in children with heart failure secondary to dilated cardiomyopathy??DCM????and to explore the correlations between RDW and the common parameters of cardiac function. Methods Totally 68 children with heart failure secondary to DCM were enrolled. According to the modified Ross scoring system??the children were divided into 3 subgroups?? mild??moderate and severe heart failure subgroups. Fifty healthy children were selected as control group. RDW levels were compared among the groups. The correlation analysis was performed between RDW and plasma N-terminal brain natriuretic peptide ??NT-proBNP??as well as left ventricular ejection fraction??LVEF??. Results RDW levels in children with heart failure secondary to DCM were significantly higher than the control group ??P??0.01??.With increasing heart failure grade??NT-proBNP levels were elevated gradually and LVEF were decreased gradually ??both P??0.01????RDW levels had no significant difference among the heart failure groups??P??0.05??. RDM was not correlated with NT-proBNP or LVEF??r??0.144??r??-0.158??P??0.05??. Conclusion In children with DCM??RDW can be used as a new diagnostic marker of heart failure??but it can not reflect the severity of heart failure.  相似文献   
3.
??Abstract??Objective To evaluate the clinical effect of transcatheter closure for ventricular septal defect in infants and young children. Methods Clinical data from 184 children under 3 years old who underwent the transcatheter VSD closure from Jan. 2011 to Dec. 2012 were analyzed. Results Procedure was successful in 176 cases ??95.7%??. No death occurred?? residual shunt in 13 cases?? tricuspid inflow accelerated in 2 cases?? late-onset complete atriovenous block in one case ??6 months after procedure??. All cases had no vascular complications. Conclusion Transcatheter closures for ventricular septal defects are effective in small children. Long-term follow-up is needed to evaluate the safety of this operation. The keys to the success of operation are the proper indications,suitable apparatus,skillful vascular punction and good anaesthetic management.  相似文献   
4.
??Abstract??Congenital hemolytic anemia ??CHA?? is a hereditary disorder with main clinical presentation of hemolytic anemia??It is the most common disease of hereditary disorders in the world. According to the pathogenic mechanism??the disorders can be divided into three groups??abnormalities of the red cell membrane??red cell enzyme disorders and hemoglobinopathies.The most common CHA are hereditary spherocytosis??G-6-PD deficiency and the thalassemias??This article focuses on the management of these three types of diseases.  相似文献   
5.
??Abstract?? Objective To evaluate effectiveness and safety of treatment for children’s acute bronchiolitis by nebulized recombinant human interferon α 1b in a multicenter clinical study??and to recommend a reasonable clinical dosage for children. Methods Withthe design of a randomized, controlled, multi-center study, totally 330 children who were hospitalized from December 2012 to May 2013 for acute bronchiolitis were randomly divided into three groups. The control group had routine and symptomatic treatment. Therapy groups inhaled human recombinant interferon α1b 2 μg/kg and 4 μg/kg respectively, twice a day plus routine treatment, for 5 to 7 days. We used four-level-scoring method to evaluate disease conditions and performed pathogen detections. Results ??1??The total improvement rates of low and high dose group were 92.3% and 95.0%, respectively, which were significantly higher than control group ??85.3%, P<0.05??. High dose group had obviously improved primary index ??wheezing, wheezing rale and three depression sign?? compared with low dose group. The disappearance time of symptoms was shortened significantly ??P<0.05??, and the improvement of three depression signs was obvious ??P<0.01??.??2??Treatments within and after 72 h were both effective, but the total improvement rate within 72 h was better.??3??The effectiveness of interferon α1b in RSV positive children was significantly better than in RSV negative children. Effectiveness of treatment group was superior to that of control group.??4??All children did not show irritation symptoms in local respiratory tracts, and no serious adverse reactions was observed. Conclusion The treatment for acute bronchiolitis in young children by nebulized human recombinant interferon α1b is effective, which shortened the duration of symptoms, with no safety problem. The effect is better when used in the early stage.  相似文献   
6.
??Abstract??Objective To explore the role of miR-125b in pediatric classical APL?? in order to seek new therapeutic strategies for drug resistant APL. Methods The target genes of miR-125b were predicted online?? validated by Dual-luciferase assay and western blot assay. MiR-125b expression levels were measured in 33 matched-pair APL samples??treated in the First Affiliated Hospital of Sun Yat-sen University and other members of South China Children APL Cooperative Group from March 2007 to September2012??at initial diagnosis and complete remission ??CR?? and in 5 relapsed patients by qRT-PCR. Proliferation and apoptosis were analyzed respectively using the RNA transfection?? MTT assay and flow cytometry. Results The expression of miR-125b was up-regulated in pediatric APL at diagnosis and relapse bone marrow samples?? but returned to normal after complete remission?? miR-125b could promote leukemic cell proliferation and inhibit cell apoptosis by regulating the expression of tumor suppressor Bak1. Remarkably??it was also found to be up-regulated in leukemic drug-resistant cells??NB4-R1??NB4-R2??HL-60/DOX???? and overexpression of exogenous miR-125b could increase their resistance to therapeutic drugs. Conclusion MiR-125b can regulate pediatric classical APL cells proliferation?? apoptosis and drug resistance by repressing BAK1 protein expression.  相似文献   
7.
??Fulminant hepatic failure refers to the severe liver function damage?? caused by multiple factors in the onset of 8 weeks of hepatic encephalopathy and severe blood coagulation dysfunction?? with onset nasty?? bad prognosis?? and high fatality rate. For the past many years,there have been no effective treatment methods. In recent years?? artificial liver support system and liver transplantation in the treatment of fulminant liver failure have more and more been taken seriously. In this paper?? the etiology?? diagnosis and treatment of FHF in recent research will be summarized.  相似文献   
8.
??The true incidence of fulminant hepatic failure in the pediatric population is unknown?? though fulminant hepatic failure is a rare but devastating syndrome??such as hepatic encephalopathy??DIC??MODS?? that results in the death of most children affected. The mainstay of the treatment is liver transplantation??however?? organ shortage limits its use.The purpose of this review is to introduce the current situation of fulminant liver failure in children??focusing on assessing the application of artificial liver??which is considered to play a pivotal role in the treatment of fulminant liver failure.According to its classification??we mainly discussed the application of non-biological artificial liver?? including its indications??advantages and disadvantages??especially the MARS. At present?? the artificial liver treatment also faces many problems. None of the ELS techniques has yet been evaluated systematically in children??and survival benefits have not yet been demonstrated.  相似文献   
9.
??Abstracts?? Objective To investigate the changes of ventricular late potentials ??VLP?? in children with vasovagal syncope of vasodepressor response ??VVS-V??. Methods Totally 184 children ??aged from 4 to 14 years old??the average age being ??10.54±2.15?? years old?? had been diagnosed with VVS-V by head-up tilt table test ??HUTT?? ??study group?? from July 2006 to January 2013 in the Second Xiangya Hospital of Central South University?? male 85 cases?? the average age of ??10.85±2.01?? years old?? female 99 cases?? mean age ??10.27±2.24?? years old. Totally 105 healthy children were matched as controls. All subjects underwent VLP examination. Investigate the difference in HR?? TQRS?? LAS40 and RMS40 between the study group and the control group. Results Compared with the control group?? in the study group heart rate ??HR?? decreased ???83.96±12.27?? beats/min vs??87.28±13.75?? beats/min?? t = -2.113?? P??0.05???the total QRS time??TQRS?? ???84.89±12.05?? ms vs ??81.21±8.23?? ms?? t = 3.070?? P??0.05?? and high frequency and low amplitude limit??LAS40?? ???62.43±19.17?? ms vs ??56.79±1.83?? ms?? t = 2.442?? P??0.05?? were prolonged??and the root mean square plant??RMS40?? increased ???28.73±7.23?? μV vs ??26.89±7.36?? μV?? t = 2.059?? P??0.05??. Conclusions Compared to healthy controls??in VVS-V children HR reduces??TQRS and LAS40 prolong??and RMS40 increases. It suggests that VVS-V childrenare more likely to have cardiovascular incidents.  相似文献   
10.
??Objective??To analyze the clinical and gene mutation characteristics of Duchenne progressive muscular dystrophy ??DMD????summarize the gene mutation hotspots in 97 cases and to explore the correlation between clinical manifestations and genotype. Methods??Totally 97 patients with DMD diagnosed by genetic examination from January 2014 to 2018 were collected and analyzed. The clinical manifestations??serum analyses and gene mutation results were analyzed. Results??The main clinical manifestations of 97 patients??96 boys?? were feeding difficulties?? increased muscle enzyme and limb weakness. Creatine kinase??CK???? lactate dehydrogenase??LDH?? and aspartate aminotransferase??AST?? muscle enzymes were significantly increased. By combining deep-sequencing technologies??the large deletions of DMD gene mutation was in 62 cases??63.92%????there were 11 cases??11.34%?? of large duplication mutation??and 24 cases??24.74%?? of point mutation. All of the mutations could occur in any position in the DMD gene??but there were two hot spots??45 cases were located in the central region gene exon 45??55??72.58% ????12 cases of deletion mutation were located in 5’exon end exon 2??19 area??19.35%??. Conclusion??The main clinical manifestations of the DMD children are feeding difficulty??increased muscle enzyme and limb weakness. The patients with significantly increased muscle enzyme should receive a timely defection of DMD gene.  相似文献   
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