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排序方式: 共有542条查询结果,搜索用时 15 毫秒
1.
Early‐Onset Heart Failure,Alopecia, and Cutaneous Abnormalities Associated with a Novel Compound Heterozygous Mutation in Desmoplakin 下载免费PDF全文
Nina K. Antonov B.S. Mina Y. Kingsbery M.D. Luis O. Rohena M.D. Teresa M. Lee M.D. Angela Christiano Ph.D. Maria C. Garzon M.D. Christine T. Lauren M.D. 《Pediatric dermatology》2015,32(1):102-108
Mutations in the desmosomal protein desmoplakin have been associated with various conditions affecting the skin and heart. The prototype is Carvajal syndrome, characterized by cardiomyopathy, woolly hair, palmoplantar keratoderma (PPK), and skin fragility. We report the case of a 3‐year‐old boy presenting with severe left‐sided heart failure with a preceding history of cutaneous abnormalities including congenital alopecia, PPK, nail dystrophy, and follicular hyperkeratosis on the extensor surfaces. Genetic testing revealed a novel combination of two heterozygous mutations in the DSP gene encoding desmoplakin: R1400X and R2284X. Both are predicted to be deleterious to protein function. This case adds to our understanding of the spectrum of clinical presentations of syndromes associated with desmoplakin mutations and highlights the need for cardiac examination in patients with characteristic cutaneous findings. 相似文献
2.
Aver’yanov A. V. Konoplyannikov A. G. Antonov N. S. Osipova G. L. Vasil’eva O. S. Sakharova M. G. Tatarskii A. R. Kobylyansky V. I. 《Bulletin of experimental biology and medicine》2018,164(4):576-578
Bulletin of Experimental Biology and Medicine - We compared survival of bone marrow mesenchymal stem cells after compressor, ultrasound, and mesh nebulization of the cell suspension over 10 min.... 相似文献
3.
Cyclosporin nephrotoxicity in heart and lung transplant patients 总被引:1,自引:0,他引:1
Griffiths MH; Crowe AV; Papadaki L; Banner NR; Yacoub MH; Thompson FD; Neild GH 《QJM : monthly journal of the Association of Physicians》1996,89(10):751-763
Twenty-two patients with heart, lung or heart and lung transplants
maintained on cyclosporin for periods ranging from 3 months to 10 years
developed renal insufficiency which was investigated by renal biopsy. The
histopathological changes were: (i) severe vascular and glomerular damage
due to thrombotic microangiopathy (TM); (ii) a form of focal segmental
glomerulosclerosis (FSGS); (iii) glomerular ischaemia. Rather than being
separate entities, these changes appeared to represent a spectrum of
pathology, some biopsies showing all three forms of glomerular injury. In
all cases the glomerular changes were accompanied by arteriolar and
arterial pathology, and we identified novel ultrastructural changes in the
arteriolar endothelial basal lamina. Tubular atrophy was a consistent
feature, the severity of which reflected the severity of the glomerular
sclerosis, and which appeared to be a consequence of glomerular loss. Our
findings are consistent with the nephrotoxic effects of cyclosporin being
mediated chiefly via damage to preglomerular vessels and glomerular
capillary endothelium. From an analysis of the clinical aspects of these
cases, the effects of cyclosporin appear to be to some extent
idiosyncratic, and therefore not entirely preventable, but strict
monitoring of blood cyclosporin levels is essential to minimize the risk of
permanent renal damage. Monitoring urinary protein in addition to plasma
creatinine may detect the onset of FSGS, as proteinuria precedes creatinine
elevation.
相似文献
4.
Ivano Amelio Vivien Landré Richard A. Knight Andrey Lisitsa Gerry Melino Alexey V. Antonov 《Oncotarget》2015,6(12):9646-9656
Targeting the ubiquitin–proteasome system (UPS) and ubiquitin-like signalling systems (UBL) has been considered a promising therapeutic strategy to treat cancer, neurodegenerative and immunological disorders. There have been multiple efforts recently to identify novel compounds that efficiently modulate the activities of different disease-specific components of the UPS-UBL. However, it is evident that polypharmacology (the ability to affect multiple independent protein targets) is a basic property of small molecules and even highly potent molecules would have a number of “off target” effects. Here we have explored publicly available high-throughput screening data covering a wide spectrum of currently accepted drug targets in order to understand polypharmacology of small molecules targeting different components of the UPS-UBL. We have demonstrated that molecules targeting a given UPS-UBL protein also have high odds to target a given off target spectrum. Moreover, the off target spectrum differs significantly between different components of UPS-UBL. This information can be utilized further in drug discovery efforts, to improve drug efficiency and to reduce the risk of potential side effects of the prospective drugs designed to target specific UPS-UBL components. 相似文献
5.
Labial adhesions are usually seen in early childhood or in the postmenopausal years, but this clinical entity is rarely seen in the reproductive years. We report a case of labial adhesion with acute urinary retention secondary to Bartholin's abscess in a reproductive‐aged woman with normal menstrual periods. We emphasize the possible occurrence of labial adhesion following Bartholin's abscess in the reproductive years with normal estrogen levels. 相似文献
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8.
Molecular characterization of a high A2 beta thalassemia by direct sequencing of single strand enriched amplified genomic DNA 总被引:2,自引:0,他引:2
Two families, one of Anglo-Saxon-Dutch descent, and the other, West Indian black, have an atypical beta thalassemia characterized by an unusually high level of Hb A2 in the heterozygous state. Restriction endonuclease mapping showed a deletion of about 1.35 kilobase (kb) in the 5' region of the beta globin gene. Direct sequencing of a specific region of genomic DNA amplified by a new modification of the polymerase chain reaction defined the deletion to be 1,393 base pairs (bp) and to be the same in both families. The deletion extends from 485 bp 5' to the mRNA CAP site to the middle of the second intervening sequence. This deletion, together with three others previously described that remove the 5' end of the beta gene but leave the delta gene intact, are all associated with unusually high levels of Hb A2 in the heterozygous state. 相似文献
9.
Beta thalassemia in Melanesia: association with malaria and characterization of a common variant (IVS-1 nt 5 G----C) 总被引:3,自引:0,他引:3
Data on the distribution of beta thalassemia among over 6,000 Melanesians reveals a major difference in the carrier rates between populations in the malarious coastal regions of New Guinea and those living in the historically malaria-free Highlands. The island of Maewo in Vanuatu has a particularly high incidence of beta + thalassemia associated with a single restriction enzyme haplotype. Direct cloning into a plasmid vector and sequence analysis demonstrate that the mutation is a G to C transversion at position 5 of intron 1 of the beta- globin gene. Oligonucleotide probe surveys indicate that this variant accounted for all cases of beta thalassemia studied from Maewo. It is also common in coastal Papua New Guinea where haplotype and oligonucleotide probe data suggest that the molecular basis of beta thalassmia is more heterogeneous. 相似文献
10.
Warkentin TE; Hayward CP; Boshkov LK; Santos AV; Sheppard JA; Bode AP; Kelton JG 《Blood》1994,84(11):3691-3699
Heparin-induced thrombocytopenia is characterized by moderate thrombocytopenia and thrombotic complications, whereas quinine/quinidine-induced thrombocytopenia usually presents with severe thrombocytopenia and bleeding. Using flow cytometry and assays of procoagulant activity, we investigated whether sera from patients with these immune drug reactions could stimulate normal platelets to generate platelet-derived microparticles with procoagulant activity. Sera or purified IgG from patients with heparin-induced thrombocytopenia stimulated the formation of platelet-derived microparticles in a heparin-dependent fashion. Further studies showed that heparin-induced thrombocytopenia sera also produced a marked increase in procoagulant activity. In contrast, sera from patients with quinine- or quinidine-induced thrombocytopenia did not generate platelet-derived microparticles nor generate increased procoagulant activity. However, quinine/quinidine-induced thrombocytopenia sera produced a significant increase in the binding of IgG to platelets in a drug-dependent fashion, whereas sera from patients with heparin-induced thrombocytopenia demonstrated no drug-dependent binding of IgG to platelets. We also observed increased levels of circulating microparticles in patients with acute heparin-induced thrombocytopenia compared with control patients. Our observations indicate that the generation of procoagulant platelet-derived microparticles in vivo is a plausible explanation for the thrombotic complications observed in some patients with heparin-induced thrombocytopenia. 相似文献