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621.
Acute graft-versus-host disease in patients with Fanconi anemia or acquired aplastic anemia undergoing bone marrow transplantation from HLA-identical sibling donors: risk factors and influence on outcome
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Guardiola P Socié G Li X Ribaud P Devergie A Espérou H Richard P Traineau R Janin A Gluckman E 《Blood》2004,103(1):73-77
To assess whether Fanconi anemia (FA) patients might be at risk for acute graft-versus-host disease (AGvHD) despite using low-intensity conditionings, we retrospectively analyzed the incidence of AGvHD and its impact on outcome in 37 FA patients and 73 patients with acquired aplastic anemia (AAA) that received transplants at Saint Louis Hospital from HLA-genotypic identical siblings with similar conditionings (thoraco-abdominal irradiation plus cyclophosphamide 20 [FA] or 150 mg/kg [AAA]). Despite being younger, FA patients had an increased risk of grades II to IV AGvHD (relative risk [RR], 2.00; P =.021), especially in younger patients (RR, 7.93; P =.014). The risks of requiring systemic corticosteroids to treat AGvHD and experiencing cortico-resistant AGvHD were significantly increased in FA patients. Although non-FA and FA patients had similar 10-year outcomes, acute and chronic GvHD had a biphasic effect on FA patient outcome with an additional cluster of lethal events starting by 5 years after transplantation. This late survival fall, restricted to FA patients, was closely related to head and neck carcinomas (15-year incidence: 53%). FA patients represent a group at risk regarding AGvHD when using irradiation-based conditionings. The impact of AGvHD on survival may not be limited to the early posttransplantation period and may be a major risk factor for head and neck carcinomas and late mortality in FA patients. 相似文献
622.
Régis Peffault de Latour Reza Tabrizi Ambroise Marcais Thierry Leblanc Thierry Lamy Mohamad Mohty Suzanne Tavitian Charlotte Jubert Marlène Pasquet Claire Galambrun Stéphanie Nguyen Jean Yves Cahn Thorsten Braun Eric Deconinck Jacques Olivier Bay Flore Sicre de Fontbrune Fiorenza Barraco Gérard Socié 《American journal of hematology》2018,93(5):635-642
Antithymocyte globulins (ATG) plus cyclosporine (CSA) is the gold standard immunosuppressive treatment (IST) for patients with aplastic anemia. A prospective randomized trial showed in 2011 that hATG was superior to rabbit ATG for first‐line treatment of severe AA. The French Health Agency (ANSM) permitted a patient‐named authorization for temporary use (ATU) program of hATG (ATGAM, Pfizer) in patients with AA in 2011 since commercial access to hATG is not approved. We took advantage of this program to analyze the outcomes of 465 patients who received antithymocyte globulins (ATGAM) plus CSA as first line treatment (n = 379; 81.5%), or for refractory (n = 26) or relapsed disease (n = 33), from September 2011 to March 2017. In the entire cohort one year, 72% of the patients had partial and 13% had complete response, with worse response for patients with severe AA and a longer interval between diagnosis and IST (more than 6 months). Severe adverse events were mainly linked to infections (24%), hemorrhages (6%), and elevated liver function tests (5%). Overall at 12 months, 9.7% of patients required second line IST and 15.6% received transplantation. Fifty‐five patients died during the study mainly because of infections (53%). Factors predicting independently worse survival were age over 40 years, neutrophils less than 0.5 × 109/L, male gender and longer delay between diagnosis and hATG (>6 months period). This study does illustrate the results of ATGAM with CSA in a true‐life perspective and confirms ATGAM as standard of care IST to treat patients with AA not eligible for HSCT. 相似文献
623.
Michael Naouri MD Serge Dahan MD Anne Le Pillouer Prost MD Phrynée Coutant-Foulc MD Catherine Raimbault MD Françoise Cucurella MD Muriel Creusot MD Martine Baspeyras MD Martine Darchy MD Randa Khallouf MD Hugues Cartier MD Isabelle Baratte MD Magali Dubois MD Hans Laubach MD Groupe de Dermatologie Esthétique et Correctrice de la Société Française de Dermatologie 《Journal of Cosmetic Dermatology》2023,22(2):342-346