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41.
Photosensitivity disorders in childhood frequently can be diagnosed and managed in the general dermatology clinic. Occasionally, when diagnostic doubt exists, referral to a specialist unit is required for diagnostic phototesting. Light testing equipment is fickle by nature, making such units uncommon. Phototesting using monochromator or provocation systems takes approximately 45 minutes. Immediate and delayed readings over the following 48 hours as appropriate are required to cover the diagnostic possibilities. Individual diseases are characterized by particular patterns of wavelength dependency and evolution of the abnormal response. Other investigations that may be required are autoantibodies to exclude lupus erythematosus, a porphyrin scan leading to full studies and, on occasion, cell mutation or survival, and chromosome studies for the rarer genophotodermatoses. Good investigative data frequently help clarify the common clinical variants. 相似文献
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AJ van Geest† PJM Berretty† PJJM Klinkhamer‡ HAM Neumann§ 《Journal of the European Academy of Dermatology and Venereology》2002,16(5):529-531
BACKGROUND: Acquired cerebriform intradermal naevus (CIN) is a rare form of pseudo cutis verticis gyrata. CASE REPORT: A case of acquired CIN of the scalp in a 46-year-old male patient is presented. The clinical and histopathological presentations of CIN are described and the therapeutic possibilities are discussed. CONCLUSIONS: In each individual case the physician must decide whether to do surgery or follow a wait-and-see policy. There is little risk of malignant change of CIN, and surgical excision of such lesions often involves evident mutilation. 相似文献
44.
Thoracic paravertebral space location 总被引:2,自引:0,他引:2
J. RICHARDSON MD MRCP FRCA S. P. S. CHEEMA FRCA J. HAWKINS FRCA S. SABANATHAN MD FRCS 《Anaesthesia》1996,51(2):137-139
45.
A E O'Hare MD FRCP G N Dutton FRCS FRCOphth D Green MB ChB MRCP Rosemary Coull 《Developmental medicine and child neurology》1998,40(6):417-420
The progress of cognitive visual dysfunction over an 8-year period of a child who sustained bilateral occipital-lobe infarctions at the age of 21/2 years is described. She survived with normal intelligence and went on to attend mainstream school. She manifested many features of cognitive visual impairment and, in particular, developed a form of pure alexia without agraphia. She achieved some letter-by-letter reading but no sight vocabulary development, including to her own name. She learned to write imaginatively employing phonetically true spelling but cannot read what she has written. Her progress and the difficulties encountered during the management of her condition are discussed in this first case report of the evolution of pure alexia without agraphia in childhood. The features of this syndrome in the developing child who has never developed the capacity to read are contrasted with that seen in affected adults. 相似文献
46.
AE Boothroyd BVS Murthy A Darbyshire AJ Petros 《Acta paediatrica (Oslo, Norway : 1992)》1996,85(12):1422-1425
Objective: Right upper lobe collapse is a common radiographic finding in intubated children. We hypothesized that deep suctioning and uncontrolled negative pressures during endotracheal tube suctioning were significant contributory factors. Methods : The incidence of right upper lobe (RUL) collapse in intubated, ventilated children on a paediatric cardiac intensive care unit was determined over a 3-month period ( n = 102). Graduated suction catheters and suction vacuums of < 165 cm H2 O were then introduced. Another prospective audit was carried out 3 months later ( n = 60). Results : We found that 24% developed RUL collapse and 4 developed an apical pneumothorax. Following the introduction of graduated catheters and controlled vacuums pressures, a significant reduction in the incidence of RUL collapse, to 7%, was observed ( p < 0.05). Conclusions : We conclude that high negative pressure and deep-suctioning causes RUL collapse in children. Any lobar collapse not only prolongs the child's stay in intensive care, but can be associated with further morbidity which may have a serious implication. By improving suctioning technique this morbidity can be significantly reduced. 相似文献
47.
Septo-optic dysplasia: MR imaging 总被引:5,自引:0,他引:5
Septo-optic dysplasia is the diagnosis when optic nerve hypoplasia is seen in conjunction with dysgenesis of the septum pellucidum. Nearly two-thirds of these patients have hypothalamic-pituitary dysfunction, and half have schizencephaly. The disorder is difficult to classify because of the diversity of clinical and pathologic manifestations. Magnetic resonance images of 11 patients with clinical and radiographic evidence of septo-optic dysplasia were reviewed retrospectively. The "syndrome" appears to include two subsets of patients whose abnormalities have different embryogenesis and neuropathologic findings. The existence of these two subsets helps to explain the diversity of the clinical and radiologic findings. 相似文献
48.
Verschuren MC; Blom B; Bogers AJ; Spits H; van Dongen JJ 《International immunology》1998,10(12):1873-1880
Recombination of deltaRec to psiJalpha will delete the TCR delta gene,
which is thought to play an important role in the bifurcation of the TCR
alphabeta versus TCR gammadelta differentiation lineages. We recently
detected a DNA-binding protein in human thymocytes, the so- called PJA-BP,
which recognizes the psiJalpha gene segment and might be one of the factors
involved in the regulation of preferential deltaRec- psiJalpha
rearrangements. We now investigate PJA-BP expression and its correlation
with TCR delta gene deletion in thymocytes. Our electrophoretic mobility
shift assay experiments showed that the PJA-BP is evolutionary conserved in
human, murine and simian thymocytes. Using a large series of human
hematopoietic malignancies (n = 30), we conclude that PJA-BP expression is
thymocyte specific and seems to be restricted to thymocytes committed to
the TCR alphabeta lineage. Analysis of seven well-defined human thymocyte
subpopulations showed that preferential deltaRec-psiJalpha rearrangements
as well as PJA-BP expression can be detected from the immature
CD34-/CD1+/CD3- /CD4+/CD8alpha+beta- thymocyte differentiation stage
onwards. These experiments indicate that expression of PJA-BP in human
thymocytes starts simultaneously with preferential deltaRec-psiJalpha
rearrangements, which supports our hypothesis that PJA-BP is one of the
factors involved in the preferential recombination of deltaRec to
psiJalpha.
相似文献
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