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排序方式: 共有443条查询结果,搜索用时 15 毫秒
1.
Fuwen Pan Qiang Liu Guoru Zhang Qiqi Wang Bo Yun Yaoguang Han Rui Deng Linqing Wu Shihua Wang 《International journal of clinical and experimental pathology》2015,8(1):948-953
Aggressive fibromatosis is a rare soft tissue tumor that composes of myofibroblasts that arise from musculoaponeurotic structures. It usually affects the abdominal wall but may be also found in other less common sites including the head and neck, submucosa of the oral cavity, spinal, haunch and limbs, especially, the limbs and sacrococcygeal region are rare locations. We described two cases of aggressive fibromatosis. One was 3-year-old girl with aggressive fibromatosis arising from the right leg region. The other was 20-year-old female arising from in the sacrococcygeal region. They were resected with satisfied results. Pathological examination showed that they were composed of fibroblasts, fibrocytes and bundles of collagen fiber. The aggressive fibromatosis, although rare, should be differentiated from some other soft tissue tumors with similar histological features and different localizations of intra-abdominal, abdominal wall and extra-abdominal. 相似文献
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Nicolas Penel Sylvie Bonvalot Marie-Cécile Le Deley Antoine Italiano Camille Tlemsani Diane Pannier Clémence Leguillette Jean-Emmanuel Kurtz Maud Toulmonde Julien Thery Daniel Orbach Pascale Dubray-Longeras Benjamin Verret François Bertucci Cécile Guillemet Lucie Laroche Armelle Dufresne Jean-Yves Blay Axel Le Cesne 《International journal of cancer. Journal international du cancer》2023,153(2):407-416
The aim of this study is to evaluate the prevalence, determinants and prognostic value of pain at diagnosis in patients with desmoid-type fibromatosis (DF). We selected patients from the ALTITUDES cohort (NCT02867033), managed by surgery, active surveillance or systemic treatments, with pain assessment at diagnosis. Patients were invited to fill QLQ-C30 questionnaire and Hospital Anxiety Depression Scale. Determinants were identified using logistic models. Prognostic value on event-free survival (EFS) was evaluated using the Cox model. Overall, 382 patients were included in the current study (median age: 40.2 years; 117 men). The prevalence of pain was 36%, without significant difference according to first-line treatment (P = .18). In the multivariate analysis, pain was significantly associated with tumor size >50 mm (P = .013) and tumor site (P < .001); pain was more frequent in the neck and shoulder locations (odds ratio: 3.05 [1.27-7.29]). Pain at baseline was significantly associated with poor quality of life (P < .001), depression (P = .02), lower performance status (P = .03) and functional impairment (P = .001); we also observed a nonsignificant association with anxiety (P = .10). In the univariate analysis, baseline pain was associated with poor EFS; the 3-year EFS was 54% in patients with pain compared to 72% in those without pain. After adjustment for sex, age, size and line of treatment, pain was still associated with poor EFS (hazard ratio: 1.82 [1.23-2.68], P = .003). One third of recently diagnosed patients with DF experienced pain, especially those with larger tumors and neck/shoulder locations. Pain was associated with unfavorable EFS after adjustment for the confounders. 相似文献
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遗传性牙龈纤维瘤病的临床研究 总被引:1,自引:0,他引:1
目的探讨遗传性牙龈纤维瘤病(hereditary gingival fibromatosis,HGF)的临床特征。方法回顾分析我院2001-2007年收治的3例HGF病例,对其遗传特点及相关的综合征,临床表现,X线片特征,组织病理,诊断分型及治疗预后进行分析。结果3例HGF患者1例为综合征型HGF,2例为非综合征型HGF,其遗传特性、发病年龄、临床表征各有所不同,但均具有典型全口牙龈增生及牙槽骨吸收。结论3例HGF不同个体表现不尽相同,具有一定的异质性。 相似文献
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Zimmermann-Laband综合征(Zimmermann-Laband syndrome,ZLS)是以发病早期口内弥漫性牙龈纤维增生为特征的一种罕见的颅颌面部畸形,伴有指甲、远节或中节趾骨发育不良或缺失。文章通过文献回顾,就ZLS的可能基因突变位点做一分析,归纳整理ZLS的临床特点、诊断、鉴别诊断及治疗方案,以期为临床诊断治疗ZLS提供依据。 相似文献
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Eellan Sivanesan & Melvin C. Gitlin 《Journal of pain & palliative care pharmacotherapy》2016,30(2):99-105
Desmoid tumors represent a nonmalignant proliferation of fibroblast-related cells. These rare tumors are difficult to treat and often persist as indolent, lifelong conditions. There are a number of treatments available for both anatomic and symptom regression. Some of these treatments, unfortunately, may not provide long-lasting results and may result in further complications. Pain is a distressing symptom that may be due to the tumor itself or the result of utilized treatments. Pharmacologic therapies represent a noninvasive alternative to surgical resection. Pain symptoms require therapeutic regimens that must be modified as the tumor evolves in expression. The individualized pain treatment program utilized may often reflect principles used in both nonmalignant and malignant pain management models. This review seeks to increase awareness of desmoid tumors through a review of the literature and discussion of its pharmacotherapeutic management. 相似文献
10.
Novel intra‐genic large deletions of CTNNB1 gene identified in WT desmoid‐type fibromatosis 下载免费PDF全文
Chiara Colombo Annalisa Astolfi Paola Collini Valentina Indio Antonino Belfiore Nicholas Paielli Federica Perrone Giuseppe Tarantino Elena Palassini Marco Fiore Andrea Pession Silvia Stacchiotti Maria Abbondanza Pantaleo Alessandro Gronchi 《Genes, chromosomes & cancer》2018,57(10):495-503
A wait and see approach for desmoid tumors (DT) has become part of the routine treatment strategy. However, predictive factors to select the risk of progressive disease are still lacking. A translational project was run in order to identify genomic signatures in patients enrolled within an Italian prospective observational study. Among 12 DT patients (10 CTNNB1‐mutated and 2 wild type) enrolled from our institution only two patients (17%) showed a progressive disease. Tumor biopsies were collected for whole exome sequencing. Overall, DT exhibited low somatic sequence mutation rate and no additional recurrent mutation was found. In the two wild type (WT) cases, two novel alterations were detected: a complex deletion of APC and a pathogenic mutation of LAMTOR2. Focusing on WT DT subtype, deep sequencing of CTNNB1, APC and LAMTOR2 was conducted on a retrospective series of 11 WT DT using a targeted approach. No other mutation of LAMTOR2 was detected, while APC was mutated in two cases. Low‐frequency (mean reads of 16%) CTNNB1 mutations were discovered in five samples (45%) and two novel intra‐genic deletions in CTNNB1 were detected in two cases. Both deletions and low frequency mutations of CTNNB1 were highly expressed. In conclusion, a minority of DT is WT for either CTNNB1, APC or any other gene involved in the WNT pathway. In this subgroup novel and hard to be detected molecular alterations in APC and CTNNB1 were discovered, contributing to explain a portion of the allegedly WT DT cases. 相似文献