全文获取类型
收费全文 | 2312篇 |
免费 | 86篇 |
国内免费 | 1篇 |
专业分类
耳鼻咽喉 | 23篇 |
儿科学 | 89篇 |
妇产科学 | 56篇 |
基础医学 | 326篇 |
口腔科学 | 140篇 |
临床医学 | 146篇 |
内科学 | 508篇 |
皮肤病学 | 114篇 |
神经病学 | 111篇 |
特种医学 | 68篇 |
外国民族医学 | 4篇 |
外科学 | 310篇 |
综合类 | 10篇 |
一般理论 | 2篇 |
预防医学 | 211篇 |
眼科学 | 29篇 |
药学 | 142篇 |
中国医学 | 5篇 |
肿瘤学 | 105篇 |
出版年
2021年 | 13篇 |
2019年 | 21篇 |
2018年 | 45篇 |
2017年 | 32篇 |
2016年 | 32篇 |
2015年 | 56篇 |
2014年 | 53篇 |
2013年 | 79篇 |
2012年 | 161篇 |
2011年 | 165篇 |
2010年 | 72篇 |
2009年 | 41篇 |
2008年 | 121篇 |
2007年 | 114篇 |
2006年 | 101篇 |
2005年 | 119篇 |
2004年 | 93篇 |
2003年 | 91篇 |
2002年 | 82篇 |
2001年 | 50篇 |
2000年 | 71篇 |
1999年 | 53篇 |
1998年 | 26篇 |
1997年 | 25篇 |
1996年 | 20篇 |
1995年 | 20篇 |
1994年 | 14篇 |
1993年 | 23篇 |
1992年 | 34篇 |
1991年 | 23篇 |
1990年 | 33篇 |
1989年 | 35篇 |
1988年 | 23篇 |
1987年 | 20篇 |
1986年 | 18篇 |
1985年 | 13篇 |
1984年 | 19篇 |
1983年 | 19篇 |
1981年 | 16篇 |
1980年 | 21篇 |
1979年 | 26篇 |
1978年 | 20篇 |
1977年 | 16篇 |
1975年 | 13篇 |
1974年 | 12篇 |
1973年 | 16篇 |
1972年 | 22篇 |
1971年 | 11篇 |
1970年 | 12篇 |
1965年 | 11篇 |
排序方式: 共有2399条查询结果,搜索用时 15 毫秒
101.
102.
103.
ALLISON BRASHEAR JONATHAN W MINK DEBORAH F HILL NIKI BOGGS W VAUGHN MCCALL MARK A STACY BEVERLY SNIVELY LANEY S LIGHT KATHLEEN J SWEADNER LAURIE J OZELIUS LESLIE MORRISON 《Developmental medicine and child neurology》2012,54(11):1065-1067
We report new clinical features of delayed motor development, hypotonia, and ataxia in two young children with mutations (R756H and D923N) in the ATP1A3 gene. In adults, mutations in ATP1A3 cause rapid‐onset dystonia–Parkinsonism (RDP, DYT12) with abrupt onset of fixed dystonia. The parents and children were examined and videotaped, and samples were collected for mutation analysis. Case 1 presented with fluctuating spells of hypotonia, dysphagia, mutism, dystonia, and ataxia at 9 months. After three episodes of hypotonia, she developed ataxia, inability to speak or swallow, and eventual seizures. Case 2 presented with hypotonia at 14 months and pre‐existing motor delay. At age 4 years, he had episodic slurred speech, followed by ataxia, drooling, and dysarthria. He remains mute. Both children had ATP1A3 gene mutations. To our knowledge, these are the earliest presentations of RDP, both with fluctuating features. Both children were initially misdiagnosed. RDP should be considered in children with discoordinated gait, and speech and swallowing difficulties. 相似文献
104.
105.
Berthold Rzany Said Hilton Welf Prager Vanessa Hartmann Gertrud Brandl Tanja C. Fischer Oliver Gekeler Simone Glöckner Gebhard Gramlich Melanie Hartmann Kathrin Lederman Jana Luckner‐Neugebauer Tatjana Pavicic Sabine Stangl Torsten Walker Sabine Zenker Marianne Wolters 《Journal der Deutschen Dermatologischen Gesellschaft》2010,8(3):210-216
106.
Gláucia Andréia Soares Guelsin Ana Maria Sell Lilian Castilho Viviane Lika Masaki Fabiano Cavalcante Melo Margareth Naomi Hashimoto Tatiana Takahashi Higa Loide Souza Hirle Jeane Eliete Laguila Visentainer 《Journal of clinical laboratory analysis》2010,24(5):311-316
We evaluated the usefulness of blood group genotyping as a supplement to hemagglutination to determine the red blood cell (RBC) antigen profile of polytransfused patients with hematological diseases and renal failure. Seventy‐nine patients were selected. They all received more than three units of blood and eight (10%) had already clinical significant alloantibodies occurring alone or in combination against Rh, K, Fya, and Di antigens. DNA was prepared from blood samples and RHCE*E/e, KEL*01/KEL*02, FY*01/FY*02 and JK*01/JK*02 alleles were determined by using PCR‐RFLP. RHD*/RHD*Ψ and RHCE*C/c were tested using multiplex PCR. Discrepancies for Rh, Kell, Duffy, and Kidd systems were found between the phenotype and genotype‐derived phenotype in 16 of the 38 chronically transfused patients. The genotypes of these patients were confirmed by DNA array analysis (HEA Beadchip?; Bioarray Solutions, Warren, NJ). Genotyping was very important for the determination of the true blood groups of the polytransfused patients, helped in the identification of suspected alloantibodies and in the selection of antigen‐negative RBCs for transfusion. J. Clin. Lab. Anal. 24:311–316, 2010. © 2010 Wiley‐Liss, Inc. 相似文献
107.
108.
109.
110.
Alexander O. Vortmeyer Eric A. Falke Sven Gläsker Jie Li Edward H. Oldfield 《Acta neuropathologica》2013,125(3):333-350
Patients with von Hippel–Lindau disease carry a germline mutation of the Von Hippel–Lindau (VHL) tumor-suppressor gene. We discuss the molecular consequences of loss of VHL gene function and their impact on the nervous system. Dysfunction of the VHL protein causes accumulation and activation of hypoxia inducible factor (HIF) which can be demonstrated in earliest stages of tumorigenesis and is followed by expression of VEGF, erythropoietin, nitric oxide synthase and glucose transporter 1 in VHL-deficient tumor cells. HIF-independent functions of VHL, epigenetic inactivation of VHL, pVHL proteostasis, and links between loss of VHL function and developmental arrest are also described. A most intriguing feature in VHL disease is the occurrence of primary hemangioblastic tumors in the nervous system, the origin of which has not yet been entirely clarified, and current hypotheses are discussed. Endolymphatic sac tumors may extend into the brain, but originally arise from proliferation of endolymphatic duct/sac epithelium; the exact nature of the proliferating epithelial cell, however, also has remained unclear, as well as the question why tumors almost consistently develop in the intraosseous portion of the endolymphatic sac/duct only. The epitheloid clear cell morphology of both advanced hemangioblastoma and renal clear cell carcinoma can make the differential diagnosis challenging, recent developments in immunohistochemical differentiation are discussed. Finally, metastasis to brain may not only be caused by renal carcinoma, but may derive from VHL disease-associated pheochromocytoma/paraganglioma, or pancreatic neuroendocrine tumor. 相似文献