全文获取类型
收费全文 | 798篇 |
免费 | 69篇 |
国内免费 | 11篇 |
专业分类
耳鼻咽喉 | 3篇 |
儿科学 | 11篇 |
妇产科学 | 1篇 |
基础医学 | 166篇 |
临床医学 | 77篇 |
内科学 | 321篇 |
皮肤病学 | 4篇 |
神经病学 | 73篇 |
特种医学 | 49篇 |
外科学 | 73篇 |
综合类 | 1篇 |
预防医学 | 21篇 |
眼科学 | 2篇 |
药学 | 42篇 |
中国医学 | 1篇 |
肿瘤学 | 33篇 |
出版年
2024年 | 4篇 |
2023年 | 7篇 |
2022年 | 11篇 |
2021年 | 20篇 |
2020年 | 18篇 |
2019年 | 32篇 |
2018年 | 26篇 |
2017年 | 20篇 |
2016年 | 27篇 |
2015年 | 30篇 |
2014年 | 31篇 |
2013年 | 37篇 |
2012年 | 70篇 |
2011年 | 84篇 |
2010年 | 31篇 |
2009年 | 33篇 |
2008年 | 45篇 |
2007年 | 51篇 |
2006年 | 49篇 |
2005年 | 37篇 |
2004年 | 45篇 |
2003年 | 34篇 |
2002年 | 34篇 |
2001年 | 4篇 |
2000年 | 2篇 |
1999年 | 4篇 |
1998年 | 11篇 |
1997年 | 10篇 |
1996年 | 11篇 |
1995年 | 11篇 |
1994年 | 8篇 |
1993年 | 6篇 |
1992年 | 3篇 |
1991年 | 3篇 |
1990年 | 4篇 |
1989年 | 3篇 |
1988年 | 4篇 |
1987年 | 2篇 |
1985年 | 1篇 |
1984年 | 1篇 |
1982年 | 2篇 |
1981年 | 1篇 |
1980年 | 1篇 |
1979年 | 1篇 |
1976年 | 3篇 |
1974年 | 2篇 |
1972年 | 1篇 |
1971年 | 1篇 |
1969年 | 1篇 |
1965年 | 1篇 |
排序方式: 共有878条查询结果,搜索用时 15 毫秒
871.
Cluzeau C Hadj-Rabia S Jambou M Mansour S Guigue P Masmoudi S Bal E Chassaing N Vincent MC Viot G Clauss F Manière MC Toupenay S Le Merrer M Lyonnet S Cormier-Daire V Amiel J Faivre L de Prost Y Munnich A Bonnefont JP Bodemer C Smahi A 《Human mutation》2011,32(1):70-72
Hypohidrotic and anhidrotic ectodermal dysplasia (HED/EDA) is a rare genodermatosis characterized by abnormal development of sweat glands, teeth, and hair. Three disease-causing genes have been hitherto identified, namely, (1) EDA1 accounting for X-linked forms, (2) EDAR, and (3) EDARADD, causing both autosomal dominant and recessive forms. Recently, WNT10A gene was identified as responsible for various autosomal recessive forms of ectodermal dysplasias, including onycho-odonto-dermal dysplasia (OODD) and Sch?pf-Schulz-Passarge syndrome. We systematically studied EDA1, EDAR, EDARADD, and WNT10A genes in a large cohort of 65 unrelated patients, of which 61 presented with HED/EDA. A total of 50 mutations (including 32 novel mutations) accounted for 60/65 cases in our series. These four genes accounted for 92% (56/61 patients) of HED/EDA cases: (1) the EDA1 gene was the most common disease-causing gene (58% of cases), (2)WNT10A and EDAR were each responsible for 16% of cases. Moreover, a novel disease locus for dominant HED/EDA mapped to chromosome 14q12-q13.1. Although no clinical differences between patients carrying EDA1, EDAR, or EDARADD mutations could be identified, patients harboring WNT10A mutations displayed distinctive clinical features (marked dental phenotype, no facial dysmorphism), helping to decide which gene should be first investigated in HED/EDA. 相似文献
872.
The case of a human immunodeficiency virus (HIV) and hepatitis C virus (HCV)-coinfected patient with a medical history of deep venous thrombosis is presented. A liver biopsy, performed for unexpected portal hypertension, showed vascular lesions presenting as nodular regenerative hyperplasia. Vascular liver diseases seem to be a new cause of chronic liver disease in HIV-infected patients receiving combined antiretroviral therapy. The syndrome of HIV-associated liver vasculopathy is discussed. 相似文献
873.
Damien Guinault Emmanuel Canet Antoine Huart Arnaud Jaccard David Ribes Laurence Lavayssiere Marion Venot Olivier Cointault Murielle Roussel Marie‐Béatrice Nogier Claire Pichereau Virginie Lemiale Bertrand Arnulf Michel Attal Dominique Chauveau Elie Azoulay Stanislas Faguer 《British journal of haematology》2016,174(6):868-875
Amyloidosis is a rare and threatening condition that may require intensive care because of amyloid deposit‐related organ dysfunction or therapy‐related adverse events. Although new multiple myeloma drugs have dramatically improved outcomes in AL amyloidosis, the outcomes of AL patients admitted into intensive care units (ICUs) remain largely unknown. Admission has been often restricted to patients with low Mayo Clinic staging and/or with a complete or very good immunological response at admission. In a retrospective multicentre cohort of 66 adult AL (n = 52) or AA (n = 14) amyloidosis patients, with similar causes of admission to an ICU, the 28‐d and 6‐month survival rates of AA patients were significantly higher compared to AL patients (93% vs. 60%, P = 0·03; 71% vs. 45%, P = 0·02, respectively). In AL patients, the simplified Index of Gravity Score (IGS2) was the only independent predictive factor for death by day 28, whereas the Mayo‐Clinic classification stage had no influence. In Cox's multivariate regression model, only cardiac arrest and on‐going chemotherapy at ICU admission significantly predicted death at 6 months. Short‐term outcomes of AL patients admitted into an ICU were mainly related to the severity of the acute medical condition, whereas on‐going chemotherapy for active amyloidosis impacted on long‐term outcomes. 相似文献
874.
Long‐term follow‐up of clinical trial patients treated for chronic HCV infection with daclatasvir‐based regimens 下载免费PDF全文
K. Rajender Reddy Stanislas Pol Paul J. Thuluvath Hiromitsu Kumada Joji Toyota Kazuaki Chayama James Levin Eric J. Lawitz Adrian Gadano Wayne Ghesquiere Guido Gerken Maurizia R. Brunetto Cheng‐Yuan Peng Marcelo Silva Simone I. Strasser Jeong Heo Fiona McPhee Zhaohui Liu Rong Yang Misti Linaberry Stephanie Noviello 《Liver international》2018,38(5):821-833
Background & Aims
Daclatasvir has achieved high sustained virologic response (SVR) rates in diverse hepatitis C virus (HCV) populations. This study evaluated the long‐term efficacy and safety of daclatasvir‐based regimens administered during clinical studies.Methods
Patients enrolled within 6 months of parent study completion or protocol availability at the study sites. The primary objective was durability of SVR at follow‐up Week 12 (SVR12). Secondary objectives included analysing HCV sequences in non‐responders or responders who relapsed, and characterization of liver disease progression.Results
Between 24 February 2012 and 17 July 2015, this study enrolled and began following 1503 recipients of daclatasvir‐based regimens (follow‐up cut‐off, 13 October 2015); 60% were male, 18% aged ≥65 years, 87% had genotype‐1a (42%) or ‐1b (45%) infection, and 18% had cirrhosis. Median follow‐up from parent study follow‐up Week 12 was 111 (range, 11‐246) weeks. 1329/1489 evaluable patients were SVR12 responders; 1316/1329 maintained SVR until their latest visit. Twelve responders relapsed by (n = 9) or after (n = 3) parent study follow‐up Week 24; one was reinfected. Relapse occurred in 3/842 (0.4%) and 9/487 (2%) responders treated with interferon‐free or interferon‐containing regimens, respectively. Hepatic disease progression and new hepatocellular carcinoma were diagnosed in 15 and 23 patients, respectively. Among non‐responders, emergent non‐structural protein‐5A (NS5A) and ‐3 (NS3) substitutions were replaced by wild‐type sequences in 27/157 (17%) and 35/47 (74%) patients, respectively.Conclusions
SVR12 was durable in 99% of recipients of daclatasvir‐based regimens. Hepatic disease progression and new hepatocellular carcinoma were infrequent. Emergent NS5A substitutions persisted longer than NS3 substitutions among non‐responders. 相似文献875.
Direct‐acting antivirals and hepatitis B virus (HBV) reactivation in co‐infected HBV/HCV kidney‐transplant recipients 下载免费PDF全文
Stanislas Pol Olivier Marion Anaïs Vallet‐Pichard Jean‐François Meritet Karine Sauné Laurent Alric Nassim Kamar 《Transplant infectious disease》2018,20(3)
Direct‐acting agents (DAAs) are highly efficient at treating hepatitis C virus (HCV) infections after kidney transplantation. Although drug agencies have recently warned of the risk of hepatitis B virus (HBV) reactivation after patients have received DAAs, reports have discrepant results in HBsAg‐positive and HBsAg‐negative patients. We report on 3 cases of HBV reactivation that were detected after achieving a DAA‐associated sustained virological response in 3 kidney‐transplant recipients initially HBsAg‐negative. In the first case, retrospective virological analysis revealed that HBsAgs had become positive and HBV DNA was detectable before initiating DAA therapy. In the second and third cases, HBV reactivation occurred 2 months and more than 1 year after stopping anti‐HCV therapy. These cases underline the discrepancies and highlight the need for comprehensive information before making definitive conclusions regarding the causal link between DAAs and HBV reactivation. 相似文献
876.
Gutkin BS Dehaene S Changeux JP 《Proceedings of the National Academy of Sciences of the United States of America》2006,103(4):1106-1111
We present a hypothetical neurocomputational model that combines a set of neural circuits at the molecular, cellular, and system levels and accounts for several neurobiological and behavioral processes leading to nicotine addiction. We propose that combining changes in the nicotinic receptor response, expressed by mesolimbic dopaminergic neurons, with dopamine-gated learning in action-selection circuits, suffices to capture the acquisition of nicotine addiction. We show that an opponent process enhanced by persistent nicotine-taking renders self-administration rigid and habitual by inhibiting the learning process, resulting in long-term impairments in the absence of the drug. The model implies distinct thresholds on the dosage and duration for the acquisition and persistence of nicotine addiction. Our hypothesis unites a number of prevalent ideas on nicotine action into a coherent formal network for further understanding of compulsive drug addiction. 相似文献
877.
878.
Octreotide treatment of chronic intestinal pseudoobstruction secondary to connective tissue diseases
Gabriel Perlemuter Patrice Cacoub Stanislas Chaussade Bertrand Wechsler Daniel Couturier Jean-Charles Piette 《Arthritis \u0026amp; Rheumatology》1999,42(7):1545-1549
Chronic intestinal pseudoobstruction (CIPO) is a rare syndrome that may occur in association with connective tissue diseases (CTD). Effective management is a major challenge. We report 3 cases in which subcutaneous octreotide was efficacious in the treatment of digestive symptoms in CIPO. In 2 of the 3 cases, previous treatment with domperidone, cisapride, or erythromycin had been unsuccessful. All 3 patients underwent a regimen of oral antibiotics along with octreotide to stimulate small bowel motility. The effects of octreotide were evident within 48 hours after the first injection in all patients. In 2, the efficacy seemed to decrease after 1 week and 6 months respectively, but increasing the dosage led to another remission. CIPO in CTD is a severe condition that can evolve regardless of the underlying disease activity. Octreotide appears to be efficacious in improving both clinical symptoms and manometric patterns. When its therapeutic effect diminishes, increasing the dosage can be useful. 相似文献