全文获取类型
收费全文 | 451篇 |
免费 | 63篇 |
国内免费 | 2篇 |
专业分类
耳鼻咽喉 | 5篇 |
儿科学 | 33篇 |
妇产科学 | 3篇 |
基础医学 | 259篇 |
口腔科学 | 6篇 |
临床医学 | 23篇 |
内科学 | 40篇 |
皮肤病学 | 6篇 |
神经病学 | 17篇 |
特种医学 | 54篇 |
外科学 | 33篇 |
综合类 | 2篇 |
预防医学 | 11篇 |
眼科学 | 7篇 |
药学 | 10篇 |
肿瘤学 | 7篇 |
出版年
2023年 | 4篇 |
2022年 | 3篇 |
2021年 | 5篇 |
2020年 | 8篇 |
2019年 | 9篇 |
2018年 | 10篇 |
2017年 | 10篇 |
2016年 | 7篇 |
2015年 | 21篇 |
2014年 | 11篇 |
2013年 | 17篇 |
2012年 | 6篇 |
2011年 | 11篇 |
2010年 | 10篇 |
2009年 | 9篇 |
2008年 | 14篇 |
2007年 | 15篇 |
2006年 | 14篇 |
2005年 | 18篇 |
2004年 | 18篇 |
2003年 | 18篇 |
2002年 | 16篇 |
2001年 | 11篇 |
2000年 | 12篇 |
1999年 | 13篇 |
1998年 | 20篇 |
1997年 | 21篇 |
1996年 | 14篇 |
1995年 | 22篇 |
1994年 | 12篇 |
1993年 | 14篇 |
1992年 | 9篇 |
1991年 | 9篇 |
1990年 | 9篇 |
1989年 | 6篇 |
1988年 | 12篇 |
1987年 | 16篇 |
1986年 | 8篇 |
1985年 | 8篇 |
1984年 | 12篇 |
1983年 | 4篇 |
1982年 | 6篇 |
1981年 | 3篇 |
1980年 | 3篇 |
1979年 | 3篇 |
1977年 | 2篇 |
1976年 | 5篇 |
1975年 | 2篇 |
1974年 | 2篇 |
1972年 | 2篇 |
排序方式: 共有516条查询结果,搜索用时 0 毫秒
511.
Contribution of congenital heart disease to neuropsychiatric outcome in school‐age children with 22q11.2 deletion syndrome 下载免费PDF全文
512.
Gaynor JW Gerdes M Zackai EH Bernbaum J Wernovsky G Clancy RR Newman MF Saunders AM Heagerty PJ D'Agostino JA McDonald-McGinn D Nicolson SC Spray TL Jarvik GP 《The Journal of thoracic and cardiovascular surgery》2003,126(6):1736-1745
BACKGROUND: There has been increasing recognition of adverse neurodevelopmental sequelae in some children after repair of congenital heart defects. Even among children with the same cardiac defect, significant interindividual variation exists in developmental outcome. Polymorphisms of apolipoprotein E have been identified as a risk factor for worse neurologic recovery after central nervous system injury. METHODS: A single-institution prospective study of patients 相似文献
513.
Background
Children with Down syndrome (DS) have about a 40 to 50% incidence of congenital heart disease (CHD). The objectives of this study were to evaluate the distribution and frequency of CHD patterns in Libyan children with DS.Methods
All patients with DS who were referred to the cardiology clinic between January 1995 and December 2008 were reviewed.Results
Of the 1 193 patients reviewed, 537 (45%) had an associated CHD. Overall there were 349 (65%) patients who had a single cardiac lesion, and 188 (35%) had multiple cardiac lesions. The most common isolated cardiac lesion was atrial septal defect (ASD), found in 125 (23%) patients, followed by atrioventricular septal defect (AVSD) in 103 (19%), and ventricular septal defect (VSD) in 76 (14%).Conclusion
Atrial septal defect was the most common cardiac lesion. The distribution of CHDs in Libyan children with DS was similar to what has been reported internationally, but the frequency was not compared with international rates. 相似文献514.
Jawad AF Prak EL Boyer J McDonald-McGinn DM Zackai E McDonald K Sullivan KE 《Journal of clinical immunology》2011,31(6):927-935
Prior to the advent of cardiac bypass, most children with congenital cardiac anomalies and chromosome 22q11.2 deletion syndrome
died. With improved technology, there is now a wave of young adults with chromosome 22q11.2 deletion syndrome requiring clinical
care. Fifteen young children and 20 adults with chromosome 22q11.2 deletion had flow cytometry, functional T cell analyses,
and functional B cell analyses to characterize their immune system. Subjects were vaccinated with the annual inactivated influenza
vaccine, and responses were evaluated by hemagglutination inhibition titer assessment. The pattern of T cell subset abnormalities
was markedly different between pediatric and adult patients. In spite of the cellular deficits observed in adults, titers
produced after influenza vaccine administration were largely intact. We conclude that disruption to T cell production appears
to have secondary consequences for T cell differentiation and B cell function although the clinical impact remains to be determined. 相似文献
515.
Burkardt DD Rosenfeld JA Helgeson ML Angle B Banks V Smith WE Gripp KW Moline J Moran RT Niyazov DM Stevens CA Zackai E Lebel RR Ashley DG Kramer N Lachman RS Graham JM 《American journal of medical genetics. Part A》2011,155(6):1336-1351
Reports of individuals with deletions of 1q24→q25 share common features of prenatal onset growth deficiency, microcephaly, small hands and feet, dysmorphic face and severe cognitive deficits. We report nine individuals with 1q24q25 deletions, who show distinctive features of a clinically recognizable 1q24q25 microdeletion syndrome: prenatal-onset microcephaly and proportionate growth deficiency, severe cognitive disability, small hands and feet with distinctive brachydactyly, single transverse palmar flexion creases, fifth finger clinodactyly and distinctive facial features: upper eyelid fullness, small ears, short nose with bulbous nasal tip, tented upper lip, and micrognathia. Radiographs demonstrate disharmonic osseous maturation with markedly delayed bone age. Occasional features include cleft lip and/or palate, cryptorchidism, brain and spinal cord defects, and seizures. Using oligonucleotide-based array comparative genomic hybridization, we defined the critical deletion region as 1.9 Mb at 1q24.3q25.1 (chr1: 170,135,865-172,099,327, hg18 coordinates), containing 13 genes and including CENPL, which encodes centromeric protein L, a protein essential for proper kinetochore function and mitotic progression. The growth deficiency in this syndrome is similar to what is seen in other types of primordial short stature with microcephaly, such as Majewski osteodysplastic primordial dwarfism, type II (MOPD2) and Seckel syndrome, which result from loss-of-function mutations in genes coding for centrosomal proteins. DNM3 is also in the deleted region and expressed in the brain, where it participates in the Shank-Homer complex and increases synaptic strength. Therefore, DNM3 is a candidate for the cognitive disability, and CENPL is a candidate for growth deficiency in this 1q24q25 microdeletion syndrome. 相似文献
516.
Children with one of two genetic disorders (chromosome 22q11.2 deletion syndrome and Turner syndrome) as well typically developing controls, participated in three cognitive processing experiments. Two experiments were designed to test cognitive processes involved in basic aspects numerical cognition. The third was a test of simple manual motor reaction time. Despite significant differences in global intellectual abilities, as measured by IQ tests, performance on the two numerical cognition tasks differed little between the two groups of children with genetic disorders. However, both performed significantly more poorly than did controls. The pattern of results are consistent with the hypothesis that impairments were not due to global intellectual ability but arose in specific cognitive functions required by different conditions within the tasks. The fact that no group differences were found in the reaction time task, despite significant differences in the standardized processing speed measure, further supports the interpretation that specific cognitive processing impairments and not global intellectual or processing speed impairments explain the pattern of results. The similarity in performance on these tasks of children with unrelated genetic disorders counters the view that numerical cognition is under any direct genetic control. Instead, our findings are consistent with the view that disturbances in foundational spatiotemporal cognitive functions contribute to the development of atypical representations and processes in the domains of basic magnitude comparison and simple numerical enumeration. 相似文献