全文获取类型
收费全文 | 312篇 |
免费 | 22篇 |
国内免费 | 1篇 |
专业分类
耳鼻咽喉 | 5篇 |
儿科学 | 2篇 |
妇产科学 | 1篇 |
基础医学 | 70篇 |
临床医学 | 16篇 |
内科学 | 129篇 |
皮肤病学 | 1篇 |
神经病学 | 6篇 |
特种医学 | 2篇 |
外科学 | 76篇 |
综合类 | 1篇 |
预防医学 | 5篇 |
眼科学 | 4篇 |
药学 | 5篇 |
肿瘤学 | 12篇 |
出版年
2023年 | 2篇 |
2022年 | 1篇 |
2021年 | 9篇 |
2020年 | 5篇 |
2019年 | 10篇 |
2018年 | 12篇 |
2017年 | 5篇 |
2016年 | 6篇 |
2015年 | 6篇 |
2014年 | 7篇 |
2013年 | 18篇 |
2012年 | 17篇 |
2011年 | 18篇 |
2010年 | 11篇 |
2009年 | 13篇 |
2008年 | 9篇 |
2007年 | 8篇 |
2006年 | 12篇 |
2005年 | 14篇 |
2004年 | 19篇 |
2003年 | 23篇 |
2002年 | 26篇 |
2001年 | 10篇 |
2000年 | 16篇 |
1999年 | 8篇 |
1998年 | 3篇 |
1997年 | 1篇 |
1996年 | 1篇 |
1994年 | 2篇 |
1993年 | 2篇 |
1992年 | 5篇 |
1991年 | 8篇 |
1990年 | 9篇 |
1989年 | 2篇 |
1988年 | 2篇 |
1987年 | 3篇 |
1986年 | 2篇 |
1985年 | 1篇 |
1984年 | 2篇 |
1983年 | 2篇 |
1981年 | 3篇 |
1980年 | 1篇 |
1979年 | 1篇 |
排序方式: 共有335条查询结果,搜索用时 15 毫秒
1.
2.
J R Regueiro P Perez-Aeiego P Aparicio C Martinez P Morales A Arnaiz-Villena 《European journal of immunology》1990,20(11):2411-2416
B lymphocytes require appropriate T lymphocyte cooperation to synthesize immunoglobulins (Ig). Such interaction presumably takes place after engagement of the T cell receptor (TcR) by antigen. The present work addresses B lymphocyte function (and phenotype) in a novel type of immunodeficiency which is characterized by a TcR expression defect. In contrast to expectations, the two affected siblings that were studied displayed normal in vivo antibody responses to both endogenous and exogenous protein antigens. However, they showed impaired responses to certain polysaccharide antigens together with a selective IgG2 deficiency. These results suggest that some polysaccharide responses may be more T cell dependent than previously suspected, and support the notion that T cell dysfunctions (of this or other kind), rather than Ig gene deletions, may be the molecular basis of certain IgG2 deficiencies. To rule out a concomitant gross B cell dysfunction in these individuals, B lymphocyte phenotype and function were assayed in vitro, and found to be normal. A T cell line derived from one of the siblings displayed an abnormal TcR on the cell surface, but it showed several normal TcR-mediated functions. This suggests that the low number of peripheral T lymphocytes that have been found to express low TcR levels in these immunodeficiencies may be operational, and supplying sufficient "help" for the observed normal antibody responses to all tested protein, but not polysaccharide, antigens. 相似文献
3.
Mohammad A. Karim Koji Suzuki Kazuyoshi Fukai Jangsuk Oh Deborah L. Nagle Karen J. Moore Ernest Barbosa Tzipora Falik‐Borenstein Alexandra Filipovich Yasushi Ishida Sirpa Kivrikko Christoph Klein Friedmar Kreuz Alex Levin Hiroaki Miyajima Jose R. Regueiro Carolyn Russo Eiichiro Uyama Outi Vierimaa Richard A. Spritz 《American journal of medical genetics. Part A》2002,108(1):16-22
Chediak‐Higashi syndrome (CHS) is a rare autosomal recessive disorder characterized by severe immunologic defects, reduced pigmentation, bleeding tendency, and progressive neurological dysfunction. Most patients present in early childhood and die unless treated by bone marrow transplantation. About 10–15% of patients exhibit a much milder clinical phenotype and survive to adulthood, but develop progressive and often fatal neurological dysfunction. Very rare patients exhibit an intermediate adolescent CHS phenotype, presenting with severe infections in early childhood, but a milder course by adolescence, with no accelerated phase. Here, we describe the organization and genomic DNA sequence of the CHS1 gene and mutation analysis of 21 unrelated patients with the childhood, adolescent, and adult forms of CHS. In patients with severe childhood CHS, we found only functionally null mutant CHS1 alleles, whereas in patients with the adolescent and adult forms of CHS we also found missense mutant alleles that likely encode CHS1 polypeptides with partial function. Together, these results suggest an allelic genotype–phenotype relationship among the various clinical forms of CHS. © 2002 Wiley‐Liss, Inc. 相似文献
4.
5.
Infliximab for treatment of pyoderma gangrenosum associated with inflammatory bowel disease 总被引:7,自引:0,他引:7
Regueiro M Valentine J Plevy S Fleisher MR Lichtenstein GR 《The American journal of gastroenterology》2003,98(8):1821-1826
OBJECTIVES: Pyoderma gangrenosum is an immune-mediated inflammatory condition characterized by ulcerative skin lesions affecting 1-2% of patients with inflammatory bowel disease (IBD). Treatment includes wound care, antibiotics, corticosteroids, and immunomodulators. However, response to therapy varies, and many patients with pyoderma gangrenosum have disease that is refractory to these agents. The aim of this study was to assess the response of medically refractory pyoderma gangrenosum to infliximab. METHODS: This was a multicenter retrospective study of patients with IBD and medically refractory pyoderma gangrenosum treated with infliximab. Data collected included the following: baseline demographics; duration of IBD; history of bowel resection; duration of skin lesions; number, size, and location of pyoderma gangrenosum lesions; prior medications; dose and number of infliximab infusions; bowel activity before and after infliximab; pyoderma gangrenosum activity before and after infliximab therapy; time to response and time to healing of pyoderma gangrenosum lesions; recurrence of pyoderma gangrenosum after infliximab; corticosteroid taper; and adverse reactions to infliximab. RESULTS: There were 13 patients with moderate to severe pyoderma gangrenosum and IBD treated with infliximab. All patients demonstrated complete healing of the skin lesions. Three patients had a complete response to induction infliximab therapy and did not require additional treatment. Ten patients responded to induction infliximab and have maintained pyoderma gangrenosum healing with infusions every 4-12 wk. All patients receiving corticosteroids were able to discontinue them completely after institution of infliximab treatment. Infliximab was well tolerated; the only treatment-related adverse events were sunburn in one patient and an infusion reaction in another. CONCLUSIONS: Infliximab is a safe and effective treatment for IBD-associated pyoderma gangrenosum. 相似文献
6.
Inflammatory bowel disease emergencies 总被引:1,自引:0,他引:1
Emergency complications of IBD are rare, but may be life-threatening, require surgery, and result in permanent end organ damage. The most common complications associated with UC are fulminant colitis, toxic megacolon, and bleeding. Each of these complications may resolve with aggressive medical therapy but often result in a total proctocolectomy. The most common complications associated with CD are abscesses and intestinal obstruction. Although initial treatment includes medical treatment, these Crohn's-related complications usually require a surgical intervention and intestinal resection. Finally, the most common extraintestinal manifestations that present as an emergency include thromboembolic events, ocular complications, and hepatobiliary disease. Some of these complications may parallel the course of the underlying disease and respond to IBD treatment, but thromboemboli, uveitis, and PSC do not. In the last decade there has been an explosion of knowledge and discovery into the pathogenesis of IBD. These findings have led to better and earlier treatment of IBD that it is hoped will alter the natural course of disease and prevent many of the complications outlined in this article. 相似文献
7.
DR, C4, and Bf allotypes in juvenile rheumatoid arthritis 总被引:1,自引:0,他引:1
A Arnaiz-Villena J J Gómez-Reino M L Gamir J R Regueiro J L Vicario F J Gómez-Reino A Alonso M P Fernandez-Dapica M V Irigoyen I Mateo 《Arthritis and rheumatism》1984,27(11):1281-1285
HLA-DR, C4, and Bf typing was performed in 99 patients with juvenile rheumatoid arthritis (JRA). DR1 was found with higher frequency in patients with polyarticular JRA than in controls (P less than 0.05). DR3 was more common in patients with fever and/or rash than in those without these manifestations (P less than 0.05). A significant negative association between JRA and C4A6 (P less than 0.05), C4BQ0 (P less than 0.0005), and BfF1 (P less than 0.05) was found. It is possible that a disequilibrium between DR and C4/Bf genes plays a role in the pathogenesis of JRA. 相似文献
8.
9.
Trasca Livia Sanchis Laura Regueiro Ander Freixa Xavier Vinereanu Dragos Sitges Marta 《The international journal of cardiovascular imaging》2021,37(5):1577-1585
The International Journal of Cardiovascular Imaging - The aim of our study was to assess the anatomical changes of the mitral valve apparatus after percutaneous repair with the MitraClip®... 相似文献
10.