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81.
Metabolic activity of phagocytosing granulocytes in chronic granulocytic leukemia: ultrastructural observation of a degranulation defect 总被引:4,自引:0,他引:4
Cramer E; Auclair C; Hakim J; Feliu E; Boucherot J; Troube H; Bernard JF; Bergogne E; Boivin P 《Blood》1977,50(1):93-106
The functional capacities of granulocytes in patients with chronic granulocytic leukemia are still a subject of controversy, probably due to the heterogeneity of the abnormalities observed from patient to patient. For a better definition of these abnormalities, 14 patients with untreated chronic granulocytic leukemia were studied. The patients were divided into three groups on the basis of the functional activities of their phagocytosing granulocytes. In four patients (group I), the granulocytes were normal in respect to particle ingestion, nitroblue tetrazolium (NBT)-stimulated reduction, cyanide-insensitive oxygen (O2) consumption, superoxide anion (O2-)-stimulated production, hydrogen peroxide (H2O2) production, and iodination. They also had a normal myeloperoxidase (MPO) content. In four patients (group III), the granulocytes were significantly defective in all of these activities. In the six remaining patients (group II), all the initial metabolic steps of the phagocytosing granulocytes (ingestion, NBT reduction, O2 consumption, O2-production, H2O2 production) were normal, as were the MPO content of the granulocytes, while iodination was strikingly decreased. These metabolic features suggested a degranulation defect which was observed ultrastructurally in the only patient studied among these six. The phagocytosing granulocytes of this patient did not degranulate and no deposits of MPO activity were seen in the phagosomes. 相似文献
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TCR gamma delta bearing lymphocyte clones with lymphokine-activated killer activity against autologous leukemic cells 总被引:1,自引:0,他引:1
Activated T lymphocytes with the T-cell receptor (TCR) gamma delta (CD3+ and TCR delta 1+) exhibit strong cytotoxic activity against the standard natural killer (NK) and lymphokine-activated killer (LAK) sensitive target cells. In order to test the cytotoxic activity of gamma delta T lymphocytes against autologous leukemic cells, 84 clones of gamma delta T lymphocytes were obtained from the peripheral blood of three acute lymphoblastic leukemia (ALL) patients. Forty-four of these T-cell clones were active against an LAK-sensitive cell line and the other 40 were active against K562, an NK target cell line. In each of the three patients, cytotoxic clones against autologous leukemic cells were obtained. Among the 84 clones, ten were able to kill autologous tumor cells, including eight that lyse the LAK-sensitive target and two with NK activity. The clones were highly cytotoxic, stable, and easily expanded in large quantity. 相似文献
84.
Ana L. Pelayo-Negro Elena Gallardo Antonio García Pascual Sánchez-Juan Jon Infante José Berciano 《Journal of neurology》2014,261(4):675-685
The objective of this study was to analyze Charcot–Marie–Tooth disease type 1A (CMT1A) evolution. We conducted a 2-year longitudinal study in 14 CMT1A patients and 14 age- and sex-matched controls. In the patients, we performed neurological examination with hand-held dynamometry, electrophysiology, and lower-limb muscle MRI, both at baseline and 2 years later, while controls were examined at baseline only. Patients’ ages ranged from 12 to 51 years. Outstanding manifestations on initial evaluation included pes cavus, areflexia, lower-limb weakness, and foot hypopallesthesia. In evaluating muscle power, good correlation was observed between manual testing and dynamometry. Compared to controls, Lunge, 10-Meter-Walking, and 9-Hole-Peg tests were impaired. Their CMT neuropathy score and functional disability scale showed that patients exhibited mild phenotype and at most slight walking difficulty. Electrophysiology revealed marked nerve conduction slowing and variable compound muscle action potential amplitude reduction. On lower-limb muscle MRI, there was distally accentuated fatty infiltration accompanied by edema in calf muscles. All these clinico-electrophysiological and imaging findings remained almost unaltered during monitoring. Using multivariate analysis, no significant predictors of progression associated to the disease were obtained. We conclude that in the 2-year period of study, CMT1A patients showed mild progression with good concordance between clinico-electrophysiological and imaging findings. 相似文献
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Pedro Luis Cepas-Guillén Isaac Pascual Eulogio Garcia Pilar Jimenez-Quevedo Alfonso Jurado-Roman Tomás Benito-González Rodrigo Estevez-Loureiro Pedro Li Dabit Arzamendi Bruno Melica Eduardo Infante de Oliveira Pedro Martín Lorenzo Felipe Fernández-Vázquez Guillermo Galeote Luis Nombela-Franco Leire Unzue Pablo Avanzas Manel Sabate Xavier Freixa 《老年心脏病学杂志》2022,19(1):90
Given the increase in life expectancy indeveloped countries,nonagenarian population will become clinically and numerically relevant in our daily routine practice in the near future.Age has been observed to exert a profound influence on the prevalence of severe mitral regurgitation (MR) in the population.[1]Mitral valve surgery remains the gold standard of care for patients with symptomatic severe MR. 相似文献
88.
Infante Jon Serrano-Cárdenas Karla M. Corral‐Juan Marc Farré Xavier Sánchez Ivelisse de Lucas Enrique M. García Antonio Martín-Gurpegui José Luis Berciano José Matilla-Dueñas Antoni 《Journal of neurology》2020,267(2):324-330
Journal of Neurology - Adolescent-onset spastic ataxia is a proposed novel phenotype in compound heterozygous carriers of an intronic mutation (c.1909 + 22G > A) in... 相似文献
89.
Maitane Garca Imanol Amayra Juan Francisco Lpez-Paz Oscar Martnez Esther Lzaro Manuel Prez Sarah Berrocoso Mohammad Al-Rashaida Jon Infante 《Cerebellum (London, England)》2020,19(3):392-400
Chiari malformation type I (CM-I) is a neurological disorder in which cerebellar tonsils are herniated through the foramen magnum into the spinal canal. A 相似文献
90.
Ester Orlandi MD Stefano Cavalieri MD Roberta Granata MD Piero Nicolai MD Paolo Castelnuovo MD Cesare Piazza MD Alberto Schreiber MD Mario Turri-Zanoni MD Pasquale Quattrone MD Rosalba Miceli MD Gabriele Infante PhD Fausto Sessa MD Carla Facco MD Giuseppina Calareso MD Nicola Alessandro Iacovelli MD Davide Mattavelli MD Alberto Paderno MD Carlo Resteghini MD Laura Deborah Locati MD Lisa Licitra MD Paolo Bossi MD 《The Laryngoscope》2020,130(4):857-865