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排序方式: 共有176条查询结果,搜索用时 31 毫秒
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Human TLR-7-, -8-, and -9-mediated induction of IFN-alpha/beta and -lambda Is IRAK-4 dependent and redundant for protective immunity to viruses 总被引:12,自引:0,他引:12
Yang K Puel A Zhang S Eidenschenk C Ku CL Casrouge A Picard C von Bernuth H Senechal B Plancoulaine S Al-Hajjar S Al-Ghonaium A Maródi L Davidson D Speert D Roifman C Garty BZ Ozinsky A Barrat FJ Coffman RL Miller RL Li X Lebon P Rodriguez-Gallego C Chapel H Geissmann F Jouanguy E Casanova JL 《Immunity》2005,23(5):465-478
Five TLRs are thought to play an important role in antiviral immunity, sensing viral products and inducing IFN-alpha/beta and -lambda. Surprisingly, patients with a defect of IRAK-4, a critical kinase downstream from TLRs, are resistant to common viruses. We show here that IFN-alpha/beta and -lambda induction via TLR-7, TLR-8, and TLR-9 was abolished in IRAK-4-deficient blood cells. In contrast, IFN-alpha/beta and -lambda were induced normally by TLR-3 and TLR-4 agonists. Moreover, IFN-beta and -lambda were normally induced by TLR-3 agonists and viruses in IRAK-4-deficient fibroblasts. We further show that IFN-alpha/beta and -lambda production in response to 9 of 11 viruses tested was normal or weakly affected in IRAK-4-deficient blood cells. Thus, IRAK-4-deficient patients may control viral infections by TLR-3- and TLR-4-dependent and/or TLR-independent production of IFNs. The TLR-7-, TLR-8-, and TLR-9-dependent induction of IFN-alpha/beta and -lambda is strictly IRAK-4 dependent and paradoxically redundant for protective immunity to most viruses in humans. 相似文献
3.
Expression of the human T cell receptor V beta repertoire. 总被引:5,自引:0,他引:5
P J Doherty C M Roifman S H Pan U Cymerman S W Ho E Thompson S Kamel-Reid A Cohen 《Molecular immunology》1991,28(6):607-612
We have used a sensitive assay, based on amplification of cDNA by the polymerase chain reaction, to determine in a variety of human tissues the relative levels of expression of the genes coding for each of the twenty families of human TcR V beta. We have determined the diversity of the expressed TcR V beta repertoire early in the development of the immune system. We have shown that the full TcR V beta repertoire is expressed early into the second trimester; the expressed repertoire is as diverse at this point, in both fetal thymus and spleen, as it is in mature thymus and peripheral blood lymphocytes. In addition the relative expression in the fetal thymus of each V beta gene is conserved to a large extent in the fetal spleen. 相似文献
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Ahmed Aziz Bousfiha Leïla Jeddane Fatima Ailal Waleed Al Herz Mary Ellen Conley Charlotte Cunningham-Rundles Amos Etzioni Alain Fischer Jose Luis Franco Raif S. Geha Lennart Hammarström Shigeaki Nonoyama Hans D. Ochs Chaim M. Roifman Reinhard Seger Mimi L. K. Tang Jennifer M. Puck Helen Chapel Luigi D. Notarangelo Jean-Laurent Casanova 《Journal of clinical immunology》2013,33(6):1078-1087
The number of genetically defined Primary Immunodeficiency Diseases (PID) has increased exponentially, especially in the past decade. The biennial classification published by the IUIS PID expert committee is therefore quickly expanding, providing valuable information regarding the disease-causing genotypes, the immunological anomalies, and the associated clinical features of PIDs. These are grouped in eight, somewhat overlapping, categories of immune dysfunction. However, based on this immunological classification, the diagnosis of a specific PID from the clinician’s observation of an individual clinical and/or immunological phenotype remains difficult, especially for non-PID specialists. The purpose of this work is to suggest a phenotypic classification that forms the basis for diagnostic trees, leading the physician to particular groups of PIDs, starting from clinical features and combining routine immunological investigations along the way. We present 8 colored diagnostic figures that correspond to the 8 PID groups in the IUIS Classification, including all the PIDs cited in the 2011 update of the IUIS classification and most of those reported since. 相似文献
7.
Mohamed Abdelhaleem MD PhD FRCP Gino R. Somers MBBS PhD FRCPA Chaim Roifman MD FRCP FCACB Stanley Read MD PhD FRCPC Oussama Abla MD 《Pediatric blood & cancer》2016,63(9):1674-1676
Primary effusion lymphoma (PEL) is a rare lymphoma that occurs more frequently in immunocompromised adults and has a poor survival. We report a 9‐year‐old female with combined immunodeficiency with an Epstein–Barr virus positive/human herpes virus 8 negative PEL‐like lymphoma. The treatment with systemic chemotherapy for non‐Hodgkin lymphoma, zidovudine, and interferon‐α failed to control disease progression. This is the first reported pediatric case of PEL‐like lymphoma. Increased diagnostic awareness and more effective treatment strategies are needed for this rare lymphoma. 相似文献
8.
Severe combined immunodeficiency (SCID) is a lethal disease unless allogeneic bone marrow transplantation (BMT), preferably
from a family related HLA identical donor (RID) is given. Previously, some patients received HLA-mismatched related donors
(MMRD) BMT, which often resulted in slow immune reconstitution and variable survival. Alternatively, HLA-matched unrelated
donors (MUD) BMT have been suggested. Recently, we have directly compared outcome of patients with SCID who received either
MMRD or MUD BMT. Survival after MUD BMT was significantly better than after MMRD BMT. Patients who received MUD BMT also had
better engraftment of donor cells and immune reconstitution. Recent reports from other centers confirm these results finding
that MUD BMT provides excellent survival and better immune reconstitution for patients with SCID. In conclusion, MUD BMT appears
vastly superior to MMRD BMT and should be offered as first choice of treatment for patients with SCID when RID is unavailable.
Presented at the First Robert A Good Society Symposium, St. Petersburg, FL 2006. 相似文献
9.
Dong Li Rebecca C. Ahrens‐Nicklas Janice Baker Vikas Bhambhani Amy Calhoun Julie S. Cohen Matthew A. Deardorff Alberto Fernández‐Jaén Benjamin Kamien Mahim Jain Fiona Mckenzie Mark Mintz Constance Motter Kirsten Niles Alyssa Ritter Curtis Rogers Maian Roifman Sharron Townshend Catherine Ward‐Melver Samantha A. Schrier Vergano 《American journal of medical genetics. Part A》2020,182(9):2058-2067
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