全文获取类型
收费全文 | 1931篇 |
免费 | 198篇 |
国内免费 | 49篇 |
专业分类
耳鼻咽喉 | 13篇 |
儿科学 | 132篇 |
妇产科学 | 40篇 |
基础医学 | 277篇 |
口腔科学 | 54篇 |
临床医学 | 148篇 |
内科学 | 481篇 |
皮肤病学 | 41篇 |
神经病学 | 160篇 |
特种医学 | 206篇 |
外科学 | 144篇 |
综合类 | 102篇 |
预防医学 | 128篇 |
眼科学 | 13篇 |
药学 | 91篇 |
中国医学 | 3篇 |
肿瘤学 | 145篇 |
出版年
2023年 | 21篇 |
2022年 | 19篇 |
2021年 | 46篇 |
2020年 | 25篇 |
2019年 | 28篇 |
2018年 | 38篇 |
2017年 | 51篇 |
2016年 | 51篇 |
2015年 | 45篇 |
2014年 | 81篇 |
2013年 | 99篇 |
2012年 | 71篇 |
2011年 | 73篇 |
2010年 | 86篇 |
2009年 | 86篇 |
2008年 | 72篇 |
2007年 | 98篇 |
2006年 | 69篇 |
2005年 | 51篇 |
2004年 | 57篇 |
2003年 | 63篇 |
2002年 | 52篇 |
2001年 | 59篇 |
2000年 | 52篇 |
1999年 | 47篇 |
1998年 | 86篇 |
1997年 | 75篇 |
1996年 | 64篇 |
1995年 | 54篇 |
1994年 | 51篇 |
1993年 | 48篇 |
1992年 | 23篇 |
1991年 | 22篇 |
1990年 | 32篇 |
1989年 | 43篇 |
1988年 | 38篇 |
1987年 | 21篇 |
1986年 | 23篇 |
1985年 | 25篇 |
1984年 | 13篇 |
1983年 | 16篇 |
1982年 | 13篇 |
1981年 | 7篇 |
1980年 | 13篇 |
1979年 | 7篇 |
1978年 | 8篇 |
1977年 | 8篇 |
1976年 | 15篇 |
1975年 | 14篇 |
1973年 | 3篇 |
排序方式: 共有2178条查询结果,搜索用时 15 毫秒
1.
Using immunologic techniques, we studied vinculin, a cytoskeletal protein associated with the membrane-skeleton of the muscle fiber. We examined muscle biopsies from five patients with Duchenne muscular dystrophy (DMD), two with Becker's muscular dystrophy (BMD), three normal human muscle samples, and four biopsies from disease control patients. All DMD patients showed patchy and low-intensity immunostain at the sarcolemma of most fibers and, by immunoblot analysis, the content of vinculin was 42 to 61% of control values. There was no significant vinculin deficiency in samples from patients with BMD and other disease controls. The data suggest that vinculin content is reduced only in muscle where dystrophin is absent or sparse. 相似文献
2.
3.
S Dimauro C T Moraes S Shanske A Lombes H Nakase S Mita H J Tritschler E Bonilla A F Miranda E A Schon 《Revue neurologique》1991,147(6-7):443-449
Thanks to recent advances in the molecular genetics of mitochondrial encephalomyopathies, we can now begin to correlate genetic lesions with biochemical defects. In the fatal infantile myopathy due to cytochrome c oxidase (COX) deficiency, an autosomal recessive condition, immunocytochemical studies have shown an isolated defect of subunit VIIa, which is 1 of the only 2 tissue-specific subunits of human COX. In muscle biopsies from patients with Kearns-Sayre syndrome, a multisystem disorder characterized by deletions of the mitochondrial DNA (mtDNA), the activities of all mitochondrial enzymes containing mtDNA-encoded subunits are decreased. The results of Northern analysis, in situ hybridization, and immunocytochemistry in muscle, and of mitochondrial protein synthesis in cultured fibroblasts suggest that partially deleted mtDNAs are transcribed but not translated, probably due to lack of indispensable tRNAs. 相似文献
4.
Acute quadriplegic myopathy: a complication of treatment with steroids, nondepolarizing blocking agents, or both. 总被引:4,自引:0,他引:4
M Hirano B R Ott E C Raps C Minetti L Lennihan N P Libbey E Bonilla A P Hays 《Neurology》1992,42(11):2082-2087
We studied two patients who were given high-dose intravenous steroid therapy and were intubated for status asthmaticus. Both became quadriplegic and wasted within 2 weeks. EMG had myopathic abnormalities. Muscle biopsy revealed severe atrophy of most muscle fibers, with disorganization of myofibrils and selective loss of thick (myosin) filaments. Immunohistologic stains for myosin isoforms confirmed the decrease or absence of this protein. Both patients clinically improved over several months. 相似文献
5.
Calciphylaxis – a topical overview 总被引:3,自引:0,他引:3
G Arseculeratne† AT Evans‡ SM Morley† 《Journal of the European Academy of Dermatology and Venereology》2006,20(5):493-502
'Calciphylaxis', a calcification syndrome associated with ischaemic cutaneous necrosis, is acquired naturally in humans in disease states. It is a life and limb-threatening complication, usually observed in patients with renal disease and secondary hyperparathyroidism, but known to occur in the absence of renal or parathyroid disease. The reported mortality rate, which ranges from 60-80%, relates to wound infection, sepsis and organ failure. It is a small-vessel vasculopathy, which is estimated to occur in about 4% of haemodialysis patients. Clinically, violaceous, reticulate areas of cutaneous necrosis and eschar may be evident, particularly in the extremities. In addition to the clinical picture, a raised calcium phosphorous product, an elevated parathyroid hormone level, radiographic evidence of vessel and soft-tissue calcification and the finding of mural calcification affecting small arteries and arterioles on histopathology help to confirm the diagnosis of this entity which generally has a poor prognosis. A high index of suspicion and an active multidisciplinary management approach, with rigorous attention to wound care and prevention of sepsis, are vital in the management of these patients. In this overview, we discuss the pathophysiology, clinical features and associations, risk factors, diagnosis and management issues relating to calciphylaxis. 相似文献
6.
Bastiaan R Klarenbeek Alexander AFA Veenhof Elly SM de Lange Willem A Bemelman Roberto Bergamaschi Piet Heres Antonio M Lacy Wim T van den Broek Donald L van der Peet Miguel A Cuesta 《BMC surgery》2007,7(1):16
Backround
Diverticulosis is a common disease in the western society with an incidence of 33–66%. 10–25% of these patients will develop diverticulitis. In order to prevent a high-risk acute operation it is advised to perform elective sigmoid resection after two episodes of diverticulitis in the elderly patient or after one episode in the younger (< 50 years) patient. Open sigmoid resection is still the gold standard, but laparoscopic colon resections seem to have certain advantages over open procedures. On the other hand, a double blind investigation has never been performed. The Sigma-trial is designed to evaluate the presumed advantages of laparoscopic over open sigmoid resections in patients with symptomatic diverticulitis. 相似文献7.
8.
9.
Giovanni Manfredi Tuan Vu Eduardo Bonilla Eric A. Schon Salvatore DiMauro Enrica Arnaudo Lee Zhang Lewis P. Rowland Michio Hirano 《Annals of neurology》1997,42(2):180-188
We identified large-scale heteroplasmic mitochondrial DNA (mtDNA) rearrangements in a 50–year-old woman with an adult-onset progressive myopathy. The predominant mtDNA abnormality was a 21.2–kb duplicated molecule. In addition, a small population of the corresponding partially deleted 4.6–kb molecule was detected. Skeletal muscle histology revealed fibers that were negative for cytochrome c oxidase (COX) activity and had reduced mtDNA-encoded COX subunits. By single-fiber polymerase chain reaction analysis, COX-negative fibers contained a low number of wild-type or duplicated mtDNA molecules (ie, nondeleted). In situ hybridization demonstrated that the abnormal fibers contained increased amounts of mtDNA compared with normal fibers and that most of the genomes were deleted. We concluded that deleted mtDNA molecules were primarily responsible for the phenotype in this patient. 相似文献
10.