全文获取类型
收费全文 | 43篇 |
免费 | 2篇 |
国内免费 | 2篇 |
专业分类
儿科学 | 10篇 |
妇产科学 | 3篇 |
基础医学 | 10篇 |
临床医学 | 10篇 |
内科学 | 7篇 |
神经病学 | 2篇 |
外科学 | 3篇 |
预防医学 | 2篇 |
出版年
2023年 | 1篇 |
2022年 | 1篇 |
2021年 | 1篇 |
2020年 | 1篇 |
2019年 | 1篇 |
2018年 | 1篇 |
2016年 | 2篇 |
2015年 | 3篇 |
2014年 | 1篇 |
2013年 | 3篇 |
2012年 | 3篇 |
2010年 | 1篇 |
2009年 | 4篇 |
2008年 | 1篇 |
2005年 | 1篇 |
2004年 | 1篇 |
2003年 | 3篇 |
2002年 | 4篇 |
2001年 | 1篇 |
1999年 | 2篇 |
1998年 | 1篇 |
1989年 | 1篇 |
1987年 | 4篇 |
1986年 | 1篇 |
1985年 | 1篇 |
1983年 | 2篇 |
1982年 | 1篇 |
排序方式: 共有47条查询结果,搜索用时 15 毫秒
1.
2.
Molecular genetic studies have pointed to a relationship between congenital lipodystrophy syndromes and some cardiac disorders. For instance, mutations in LMNA cause either lipodystrophy or cardiomyopathy, indicating that different mutations in the same gene can produce these clinical syndromes. The present authors describe a 10-year-old female with Berardinelli-Seip congenital complete lipodystrophy (MIM 606158) caused by homozygosity for a frameshift mutation in BSCL2. In addition to the typical attributes of complete lipodystrophy, this subject had hypertrophic cardiomyopathy diagnosed in the first year of her life; its progress has been followed with non-invasive imaging. The mechanism underlying the hypertrophic cardiomyopathy in complete lipodystrophy is unclear. It may result from a direct effect of the mutant gene or it might be secondary to the effects of hyperinsulinemia on cardiac development. The variability of the associated cardiomyopathy in patients with complete generalized lipodystrophy may be caused by differential effects of mutations in the same gene or of mutations in different genes which underlie the lipodystrophy phenotype. 相似文献
3.
Age Progression of Neuropathological Markers in the Brain of the Chilean Rodent Octodon degus,a Natural Model of Alzheimer's Disease
下载免费PDF全文
![点击此处可从《Brain pathology (Zurich, Switzerland)》网站下载免费的PDF全文](/ch/ext_images/free.gif)
Nibaldo C. Inestrosa Juvenal A. Ríos Pedro Cisternas Cheril Tapia‐Rojas Daniela S. Rivera Nady Braidy Juan M. Zolezzi Juan A. Godoy Francisco J. Carvajal Alvaro O. Ardiles Francisco Bozinovic Adrián G. Palacios Perminder S. Sachdev 《Brain pathology (Zurich, Switzerland)》2015,25(6):679-691
Alzheimer's disease (AD) is the most common neurodegenerative disorder and the leading cause of age‐related dementia worldwide. Several models for AD have been developed to provide information regarding the initial changes that lead to degeneration. Transgenic mouse models recapitulate many, but not all, of the features of AD, most likely because of the high complexity of the pathology. In this context, the validation of a wild‐type animal model of AD that mimics the neuropathological and behavioral abnormalities is necessary. In previous studies, we have reported that the Chilean rodent Octodon degus could represent a natural model for AD. In the present work, we further describe the age‐related neurodegeneration observed in the O. degus brain. We report some histopathological markers associated with the onset progression of AD, such as glial activation, increase in oxidative stress markers, neuronal apoptosis and the expression of the peroxisome proliferative‐activated receptor γ coactivator‐1α (PGC‐1α). With these results, we suggest that the O. degus could represent a new model for AD research and a powerful tool in the search for therapeutic strategies against AD. 相似文献
4.
V. Phan T. Blydt-Hansen J. Feber N. Alos S. Arora S. Atkinson L. Bell C. Clarson R. Couch E. A. Cummings G. Filler R. M. Grant J. Grimmer D. Hebert B. Lentle J. Ma M. Matzinger J. Midgley M. Pinsk C. Rodd N. Shenouda R. Stein D. Stephure S. Taback K. Williams F. Rauch K. Siminoski L. M. Ward 《Osteoporosis international》2014,25(2):627-637
Summary
Incident vertebral fractures and lumbar spine bone mineral density (BMD) were assessed in the 12 months following glucocorticoid initiation in 65 children with nephrotic syndrome. The incidence of vertebral fractures was low at 12 months (6 %) and most patients demonstrated recovery in BMD Z-scores by this time point.Introduction
Vertebral fracture (VF) incidence following glucocorticoid (GC) initiation has not been previously reported in pediatric nephrotic syndrome.Methods
VF was assessed on radiographs (Genant method); lumbar spine bone mineral density (LS BMD) was evaluated by dual-energy X-ray absorptiometry.Results
Sixty-five children were followed to 12 months post-GC initiation (median age, 5.4 years; range, 2.3–17.9). Three of 54 children with radiographs (6 %; 95 % confidence interval (CI), 2–15 %) had incident VF at 1 year. The mean LS BMD Z-score was below the healthy average at baseline (mean ± standard deviation (SD), ?0.5?±?1.1; p?=?0.001) and at 3 months (?0.6?±?1.1; p?<?0.001), but not at 6 months (?0.3?±?1.3; p?=?0.066) or 12 months (?0.3?±?1.2; p?=?0.066). Mixed effect modeling showed a significant increase in LS BMD Z-scores between 3 and 12 months (0.22 SD; 95 % CI, 0.08 to 0.36; p?=?0.003). A subgroup (N?=?16; 25 %) had LS BMD Z-scores that were ≤?1.0 at 12 months. In these children, each additional 1,000 mg/m2 of GC received in the first 3 months was associated with a decrease in LS BMD Z-score by 0.39 at 12 months (95 % CI, ?0.71 to ?0.07; p?=?0.017).Conclusions
The incidence of VF at 1 year was low and LS BMD Z-scores improved by 12 months in the majority. Twenty-five percent of children had LS BMD Z-scores ≤?1.0 at 12 months. In these children, LS BMD Z-scores were inversely associated with early GC exposure, despite similar GC exposure compared to the rest of the cohort. 相似文献5.
Hypoxemia occurred after induction of anesthesia and repositioning in a patient undergoing hip pinning. The patient had previously presented to the emergency department with multiple fractures and hemodynamic instability sustained in a motor vehicle accident. Three days after admission to the intensive care unit the patient remained intubated with respiratory insufficiency and had developed acute respiratory distress syndrome with marginal oxygen saturation. The patient was transported to the operating room for hip pinning, and anesthesia was induced with midazolam, fentanyl, vecuronium, and isoflurane. When the patient was turned to the left lateral position, oxygen saturation suddenly worsened from 94% to 78%, with Pao2 from arterial blood gas measured at 54 mm Hg. The patient was returned to the supine position, but despite maneuvers to improve oxygen saturation, the patient's saturation remained below 87% and pulmonary thromboembolism was suspected. However, other signs of pulmonary embolus such as hemodynamic deterioration and right ventricular dysfunction were not present. Chest radiographs demonstrated severe left lung atelectasis, and surgery was postponed. Upon return to the intensive care unit, fiberoptic bronchoscopy was performed, and a large mucous plug was removed from his left upper and lower lobes, with subsequent improvement of Pao2 to 77 mm Hg with an oxygen saturation of 94%. 相似文献
6.
Fibrinogen binds from aqueous media containing it to droplets of linear trimethylsilyl-terminated poly(dimethylsiloxane) (PDMS) dispersed in those same media. Once bound, fibrinogen elutes from emulsified droplets of PDMS only very slowly, even when incubated in buffer that contains a physiologic concentration of the protein. The bound fibrinogen is coagulable, as indicated by the thrombin-dependent agglutination of droplets. Thus fibrinogen bound to droplets of PDMS renders an adhesive potential to the surface of the droplets, a potential that may have relevance to the biologic processing of the polymer in vivo. 相似文献
7.
D Daneman L Fishman C Clarson 《Clinical and investigative medicine. Médecine clinique et experimentale》1987,10(5):480-483
We measured insulin antibody binding in 2 groups of patients: Study 1, 32 children with newly diagnosed IDDM before onset of insulin therapy, and, in 20 of these, 10 days, 1, 3, and 6 months after beginning therapy; and Study 2, 35 children with long-standing IDDM, 20 of whom had free insulin concentrations measured before, and for 2 hours following subcutaneous injection of 0.25 U/kg regular insulin. Almost 35% of new onset subjects had insulin antibody binding above control levels. In those studied prospectively, binding increased significantly with time. Pre-treatment binding did not correlate with later insulin antibody binding nor metabolic control. In Study 1 we have confirmed previous studies showing abnormally high insulin antibody binding in children with IDDM pre-treatment. We have been unable to demonstrate a relationship between this binding and that found 6 months after initiation of therapy. In Study 2, we have shown that insulin antibody binding is not related to either the level of metabolic control or the rate of rise of free insulin levels in children with IDDM. 相似文献
8.
9.
Residual beta-cell function in children with IDDM: reproducibility of testing and factors influencing insulin secretory reserve 总被引:1,自引:0,他引:1
Reproducibility of C-peptide secretion was assessed in 20 children (group 1) by their responses to two Sustacal- (a mixed liquid meal) stimulation tests performed 7-14 days apart. For the 12 C-peptide-positive children (basal C-peptide greater than or equal to 0.03 pmol/ml) there were no differences in the basal or stimulated values between tests 1 and 2. The effect of exogenous insulin on C-peptide secretion was assessed in 20 other children (group 2) by their responses to two Sustacal tests, one test without and one with soluble insulin (0.25 U/kg) injected subcutaneously before testing. Eleven children were C-peptide positive and had no differences in C-peptide response between tests 1 and 2. The results from test 1 in groups 1 and 2 were combined with those from 44 others undergoing a single Sustacal test (group 3, N = 84). There was a close correlation between basal and peak C-peptide concentrations in the 44 C-peptide-positive children (r = .88, P less than .001). Peak C-peptide concentrations correlated inversely with HbA1 (r = -.29, P less than .01), insulin dose in units per kilogram (r = -.40, P less than .001), and duration of diabetes (r = .33, P less than .001) and positively with age at onset of diabetes (r = .34, P less than .001). The C-peptide-positive children had reduced glucose response to Sustacal, lower HbA1 concentration, lower insulin requirement, later age of onset, and shorter duration of diabetes than children who were C-peptide negative. 相似文献
10.
J. Feber I. Gaboury A. Ni N. Alos S. Arora L. Bell T. Blydt-Hansen C. Clarson G. Filler J. Hay D. Hebert B. Lentle M. Matzinger J. Midgley D. Moher M. Pinsk F. Rauch C. Rodd N. Shenouda K. Siminoski L. M. Ward 《Osteoporosis international》2012,23(2):751-760