全文获取类型
收费全文 | 792篇 |
免费 | 27篇 |
国内免费 | 5篇 |
专业分类
耳鼻咽喉 | 1篇 |
儿科学 | 32篇 |
妇产科学 | 5篇 |
基础医学 | 76篇 |
口腔科学 | 16篇 |
临床医学 | 53篇 |
内科学 | 277篇 |
皮肤病学 | 7篇 |
神经病学 | 49篇 |
特种医学 | 23篇 |
外科学 | 168篇 |
综合类 | 5篇 |
预防医学 | 13篇 |
眼科学 | 2篇 |
药学 | 50篇 |
中国医学 | 1篇 |
肿瘤学 | 46篇 |
出版年
2022年 | 7篇 |
2021年 | 10篇 |
2019年 | 6篇 |
2018年 | 11篇 |
2017年 | 9篇 |
2016年 | 6篇 |
2014年 | 11篇 |
2013年 | 12篇 |
2012年 | 25篇 |
2011年 | 20篇 |
2010年 | 12篇 |
2009年 | 13篇 |
2008年 | 22篇 |
2007年 | 26篇 |
2006年 | 21篇 |
2005年 | 33篇 |
2004年 | 20篇 |
2003年 | 30篇 |
2002年 | 30篇 |
2001年 | 27篇 |
2000年 | 35篇 |
1999年 | 28篇 |
1998年 | 14篇 |
1997年 | 12篇 |
1996年 | 13篇 |
1995年 | 9篇 |
1994年 | 11篇 |
1993年 | 8篇 |
1992年 | 24篇 |
1991年 | 20篇 |
1990年 | 21篇 |
1989年 | 28篇 |
1988年 | 24篇 |
1987年 | 18篇 |
1986年 | 32篇 |
1985年 | 14篇 |
1984年 | 15篇 |
1983年 | 11篇 |
1981年 | 11篇 |
1979年 | 11篇 |
1978年 | 7篇 |
1977年 | 11篇 |
1976年 | 7篇 |
1975年 | 9篇 |
1974年 | 11篇 |
1969年 | 14篇 |
1968年 | 5篇 |
1967年 | 7篇 |
1966年 | 6篇 |
1965年 | 5篇 |
排序方式: 共有824条查询结果,搜索用时 15 毫秒
1.
2.
Kunihiro Ichinose Mitsuru Nakamura Kenji Takezawa Ichiro Masutomi Yoichi Shima Yoko Hirayama Kahoko Sorimachi Teruhiko Shimizu Hiroyo Ishikawa Namiko Kaji Sayaka Nakajima Michiko Wataru Shiho Nishigaki Hiroshi Suwa Yosuke Toyama Masaki Okumura Yoshikazu Ishitsuka Ken Shimizu Kazuya Kokubo Kenji Sasaki Shodai Saito 《Seishin shinkeigaku zasshi》2006,108(9):945-954
3.
4.
Masashi Kato Shinji Sato Misako Suzuki Hiroko Oka Yuko Kaneko Hidekata Yasuoka Takaki Nojima Akira Suwa Michito Hirakata Yasuo Ikeda 《Nihon Rinshō Men'eki Gakkai kaishi》2004,27(5):345-349
A 52 year-old woman noticed general fatigue, polyarthralgia, and muscle weakness of lower extremities in October 2001. In December, she felt difficulty in walking due to muscle weakness. In January 2002, she admitted another hospital because of dyspnea on exertion and edema of lower extremities. Laboratory test revealed leukocytopenia, the elevation of creatine kinase and positive anti-U1-RNP antibodies. Her chest computed tomography (CT) showed severe interstitial pneumonia. Cardiac echogram revealed that she had pericardial effusion and pulmonary hypertension. Then she was transferred to Keio University Hospital and she was diagnosed as having mixed connective tissue disease (MCTD) manifestating myositis, interstitial pneumonia, pulmonary hypertension and pericarditis. Prednisolone (PSL) 60mg daily following to methylprednisolone (mPSL) pulse therapy was begun and her symptoms were gradually improved. In middle of February, she complained of high fever over 39.0 degrees C. Bacterial culture tests were negative and laboratory data indicated pancytopenia and a high level of serum ferritin. Bone marrow aspiration revealed hemophagocytosis in bone marrow specimens and she was diagnosed as having hemophagocytic syndrome associated with MCTD. mPSL pulse therapy was not effective and intermittent cyclophosphamide pulse therapy (IV-CY) was performed resulting in improvement of the symptoms. This case suggested the effectiveness of IV-CY therapy in patients with corticosteroid-resistant HPS associated with connective tissue diseases. 相似文献
5.
Spontaneous puberty in 46,XX subjects with congenital lipoid adrenal hyperplasia. Ovarian steroidogenesis is spared to some extent despite inactivating mutations in the steroidogenic acute regulatory protein (StAR) gene. 下载免费PDF全文
K Fujieda T Tajima J Nakae S Sageshima K Tachibana S Suwa T Sugawara J F Strauss rd 《The Journal of clinical investigation》1997,99(6):1265-1271
Congenital lipoid adrenal hyperplasia (lipoid CAH) is the most severe form of CAH in which the synthesis of all gonadal and adrenal cortical steroids is markedly impaired. We report here the clinical, endocrinological, and molecular analyses of two unrelated Japanese kindreds of 46,XX subjects affected with lipoid CAH who manifested spontaneous puberty. Phenotypic female infants with 46,XX karyotypes were diagnosed with lipoid CAH as newborns based on a clinical history of failure to thrive, hyperpigmentation, hyponatremia, hyperkalemia, and low basal values of serum cortisol and urinary 17-hydroxycorticosteroid and 17-ketosteroid. These patients responded to treatment with glucocorticoid and 9alpha-fludrocortisone. Spontaneous thelarche occurred in association with increased serum estradiol levels at the age of 10 and 11 yr, respectively. Pubic hair developed at the age of 12 yr 11 mo in one subject and menarche was at the age of 12 yr in both cases. Both subjects reported periodic menstrual bleeding and subsequently developed polycystic ovaries. To investigate the molecular basis of the steroidogenic lesion in these patients, the StAR gene was characterized by PCR and direct DNA sequence analyses. DNA sequence analysis revealed that one patient is homozygous for the Gln 258 Stop mutation in exon 7 and that the other patient is a compound heterozygote with the Gln 258 Stop mutation and a single A deletion at codon 238 in the other allele causing a frame-shift, which renders the StAR protein nonfunctional. These findings demonstrate that ovarian steroidogenesis can be spared to some extent through puberty when the StAR gene product is inactive. This is in marked contrast to the early onset of severe defects in testicular and adrenocortical steroidogenesis which are characteristics of this disease. 相似文献
6.
Terashima Y Onai N Murai M Enomoto M Poonpiriya V Hamada T Motomura K Suwa M Ezaki T Haga T Kanegasaki S Matsushima K 《Nature immunology》2005,6(8):827-835
Ligation of the chemokine receptor CCR2 on monocytes and macrophages with its ligand CCL2 results in activation of the cascade consisting of phosphatidylinositol-3-OH kinase (PI(3)K), the small G protein Rac and lamellipodium protrusion. We show here that a unique clathrin heavy-chain repeat homology protein, FROUNT, directly bound activated CCR2 and formed clusters at the cell front during chemotaxis. Overexpression of FROUNT amplified the chemokine-elicited PI(3)K-Rac-lamellipodium protrusion cascade and subsequent chemotaxis. Blocking FROUNT function by using a truncated mutant or antisense strategy substantially diminished signaling via CCR2. In a mouse peritonitis model, suppression of endogenous FROUNT markedly prevented macrophage infiltration. Thus, FROUNT links activated CCR2 to the PI(3)K-Rac-lamellipodium protrusion cascade and could be a therapeutic target in chronic inflammatory immune diseases associated with macrophage infiltration. 相似文献
7.
T Tamura Y Kuroki S Nagafuchi S Suwa Y Nakahori K Terashima T Furusho Y Nakagome 《The Japanese journal of human genetics》1991,36(2):195-199
A female patient with unilateral gonadal dysgenesis was a mosaic for three cell lines, 45,X/46,X, + marI/46,X, + marII, including two different marker chromosomes. DNA analysis using 17 Y-specific DNA probes revealed that each marker consists of different segments of the Y chromosome. 相似文献
8.
9.
Terada Y Imoto I Nagai H Suwa K Momoi M Tajiri T Onda M Inazawa J Emi M 《American journal of medical genetics》2001,103(2):176-180
We performed molecular analysis of a germline interstitial deletion of chromosome 4 [del(4)(q21.22q23)], which had been observed in a male infant manifesting early-onset hepatoblastoma (HBL). The chromosomal anomaly in this child was associated with a unique congenital syndrome including HBL, atrial septal defect, ventricular septal defect, patent ductus arteriosus, mental retardation, and seizures. However, the patient did not exhibit a megalencephaly typical of 4q21-22 deletions. His HBL was associated with an increasing serum alpha-fetoprotein level and rapid growth. To define the chromosomal deletion at the molecular level in this child, we analyzed his lymphoblasts with fluorescence in situ hybridization, using as probes a panel of BAC/PAC genomic clones containing STS markers covering the 4q12-27 region. The analysis revealed that the affected chromosome had an 8-cM deletion within 4q21-q22, flanked by markers D4S2964 and D4S2966. This microdeletion overlaps with the commonly deleted region at 4q21-q22 that was recently defined in adult hepatocellular carcinomas. 相似文献
10.
One-day-old chicks, inoculated intramuscularly with the MSB1-TK5803 strain of chicken anaemia agent (CAA), showed a decrease of haematocrit value and inhibition of body weight gain, particularly between days 12 and 20 post inoculation (pi). Macroscopically, yellowish bone marrow, atrophy of the thymus and bursa of Fabricius, enlargement and discoloration of liver were observed in most chicks at the peak of infection. Histologically, the lesions appeared first in bone marrow and thymus on day 6 pi, and then in the bursa, spleen and liver. Swelling and intranuclear inclusion bodies in the haematopoietic precursor cells, thymocytes and reticular cells of the thymus were considered to be characteristic lesions. Hypoplasia or aplasia of bone marrow, depletion of lymphocytes in the lymphoid organs and swelling of hepatocytes occurred during the anaemic phase. Repopulation of the haematopoietic cells and lymphocytes occurred between days 16 and 24 pi, and most tissues of the surviving chicks recovered by day 32 pi. 相似文献