全文获取类型
收费全文 | 1170篇 |
免费 | 62篇 |
国内免费 | 11篇 |
专业分类
耳鼻咽喉 | 1篇 |
儿科学 | 118篇 |
妇产科学 | 11篇 |
基础医学 | 109篇 |
口腔科学 | 36篇 |
临床医学 | 179篇 |
内科学 | 295篇 |
皮肤病学 | 15篇 |
神经病学 | 19篇 |
特种医学 | 215篇 |
外科学 | 77篇 |
综合类 | 35篇 |
预防医学 | 52篇 |
眼科学 | 18篇 |
药学 | 32篇 |
1篇 | |
肿瘤学 | 30篇 |
出版年
2021年 | 3篇 |
2020年 | 7篇 |
2019年 | 7篇 |
2018年 | 11篇 |
2017年 | 11篇 |
2016年 | 9篇 |
2015年 | 22篇 |
2014年 | 21篇 |
2013年 | 20篇 |
2012年 | 23篇 |
2011年 | 29篇 |
2010年 | 43篇 |
2009年 | 46篇 |
2008年 | 30篇 |
2007年 | 16篇 |
2006年 | 19篇 |
2005年 | 21篇 |
2004年 | 22篇 |
2003年 | 21篇 |
2002年 | 27篇 |
2001年 | 18篇 |
2000年 | 18篇 |
1999年 | 23篇 |
1998年 | 83篇 |
1997年 | 85篇 |
1996年 | 91篇 |
1995年 | 59篇 |
1994年 | 55篇 |
1993年 | 45篇 |
1992年 | 24篇 |
1991年 | 23篇 |
1990年 | 14篇 |
1989年 | 38篇 |
1988年 | 31篇 |
1987年 | 40篇 |
1986年 | 19篇 |
1985年 | 32篇 |
1984年 | 23篇 |
1983年 | 23篇 |
1982年 | 17篇 |
1981年 | 17篇 |
1980年 | 10篇 |
1979年 | 4篇 |
1978年 | 6篇 |
1977年 | 9篇 |
1976年 | 9篇 |
1975年 | 8篇 |
1971年 | 2篇 |
1969年 | 2篇 |
1966年 | 2篇 |
排序方式: 共有1243条查询结果,搜索用时 15 毫秒
31.
Hendrickx J; Dams E; Coucke P; Lee P; Fernandes J; Willems PJ 《Human molecular genetics》1996,5(5):649-652
X-linked liver glycogenosis type II (XLG II) is a recently described X-
linked liver glycogen storage disease, mainly characterized by enlarged
liver and growth retardation. These clinical symptoms are very similar to
those of XLG I. In contrast to XLG I patients, however, XLG II patients do
not show an in vitro enzymatic deficiency of phosphorylase kinase (PHK).
Recently, mutations were identified in the gene encoding the liver alpha
subunit of PHK (PHKA2) in XLG I patients. We have now studied the PHKA2
gene of four unrelated XLG II patients and identified four different
mutations in the open reading frame, including a deletion of three
nucleotides, an insertion of six nucleotides and two missense mutations.
These results indicate that XLG II is due to mutations in PHKA2. In
contrast to XLG I, XLG II is caused by mutations that lead to minor
structural abnormalities in the primary structure of the liver alpha
subunit of PHK. These mutations are found in a conserved RXX(X)T motif,
resembling known phosphorylation sites that might be involved in the
regulation of PHK. These findings might explain why the in vitro PHK
enzymatic activity is not deficient in XLG II, whereas it is in XLG I.
相似文献
32.
Wootton PT Arora NL Drenos F Thompson SR Cooper JA Stephens JW Hurel SJ Hurt-Camejo E Wiklund O Humphries SE Talmud PJ 《Human molecular genetics》2007,16(12):1437-1444
Animal and human studies suggest that both secretory PLA2 (sPLA2)-V and sPLA2-IIA (encoded, respectively, by the neighbouring PLA2G5 and PLA2G2A genes) contribute to atherogenesis. Elevated plasma sPLA2-IIA predicts coronary heart disease (CHD) risk, but no mass assay for sPLA2-V is available. We previously reported that tagging single nucleotide polymorphism (tSNP) haplotypes of PLA2G2A are strongly associated with sPLA2-IIA mass, but not lipid levels. Here, we use tSNPs of the sPLA2-V gene to investigate the association of PLA2G5 with CHD risk markers. Seven PLA2G5 tSNPs genotypes, explaining >92% of the locus genetic variability, were determined in 519 patients with Type II diabetes (in whom PLA2G2A tSNP data was available), and defined seven common haplotypes (frequencies >5%). PLA2G5 and PLA2G2A tSNPs showed linkage disequilibrium (LD). Compared to the common PLA2G5 haplotype, H1 (frequency 34.9%), haplotypes H2-7 were associated with overall higher plasma LDL (P < 0.00004) and total cholesterol (P < 0.00003) levels yet lower oxLDL/LDL (P = 0.006) and sPLA2-IIA mass (P = 0.04), probably reflecting LD with PLA2G2A. Intronic tSNP (rs11573248), unlikely itself to be functional, distinguished H1 from LDL-raising haplotypes and may mark a functional site. In conclusion, PLA2G5 tSNP haplotypes demonstrate an association with total and LDL cholesterol and oxLDL/LDL, not seen with PLA2G2A, thus confirming distinct functional roles for these two sPLA2s. 相似文献
33.
34.
Accurate pregnancy dating is vital to obstetric management. However, first trimester fetal charts commonly used in Australia rely on data reported more than three decades ago. This study reports first trimester dating and growth charts for crown‐rump length between 5 and 14 weeks of gestation and biparietal diameter between 9 and 14 weeks of gestation on an Australia population using modern real‐time ultrasound equipment. All consenting eligible women attending a large Sydney clinic for first trimester ultrasound between March 2005 and December 2006 were recruited. Measurements were carried out to Australasian Society for Ultrasound in Medicine standard protocols. Statistical analyses were undertaken using polynomial regression models and thorough diagnostic checks made. Overall 396 eligible women consented to the study, with 268 between 9 and 14 weeks of gestation. The average participant age was 34 years (range 22–45 years), 371 and all yielded valid biometry measurements. Equations, means and 90% reference intervals for crown‐rump length measurements and biparietal diameter measurements were derived using polynomial regression models. Thorough residual and diagnostic checks were made. Once validated by others, we believe they will warrant consideration for use by Australasian Society for Ultrasound in Medicine. 相似文献
35.
ÅM Jakobson A Kreuger Ö Mortimer S Henningsson H Seidel PJ Moe 《Acta paediatrica (Oslo, Norway : 1992)》1992,81(4):359-361
Two patients aged 11 and four years, were accidentally given a 10-fold overdose of intrathecal methotrexate while being treated for malignant disease. Neither patient developed any signs of neurotoxicity and exchange of lumbar cerebro-spinal fluid was started 3 and 5 h later, respectively. In one of the patients, who received 120 mg of methotrexate intrathecally, 31% of the given dose was recovered during 2 h of cerebrospinal fluid exchange that was started 3 h after the accidental overdosage. No sequelae were observed in any of the patients. Cerebrospinal fluid exchange is safe and can be recommended in all cases of intrathecal methotrexate overdosage. Ventriculo-cisternal perfusion is not necessary in cases of a 10-fold overdose if the patient has no signs of acute neurotoxicity. 相似文献
36.
WM Bisset P Stapleford S Long A Chamberlain B Sokel PJ Milla 《Archives of disease in childhood》1992,67(1):109-114
In children with severe failure of intestinal function, intravenous nutrition is at present the only treatment able to maintain adequate nutrition for prolonged periods of time. Over the last five years we have discharged 10 patients home on parenteral nutrition for a total of 25 patient years and here the outcome of these children is presented. Of the 10 patients, one has discontinued home parenteral nutrition (HPN), seven patients remain well, one patient has recently moved to the USA, and one patient has died after major abdominal surgery. All children had either normal or an accelerated rate of growth on HPN and developmentally all have progressed well. All the children over 5 years attend normal schools. The major complication of treatment was line sepsis with an overall rate of one episode in 476 days and a total of nine central lines (five patients) have required replacement giving an average line life of 680 days. For those children unfortunate enough to suffer from severe intestinal failure, HPN is preferable to prolonged hospital treatment and offers the chance of a good quality of life with prolonged survival. 相似文献
37.
JN HANNA WL SEXTON JL FAOAGALI PJ BUDA ML KENNETT KA BRUSSEN 《Journal of paediatrics and child health》1995,31(4):345-349
Objective: To determine the immunity to hepatitis B, poliomyelitis and measles in fully vaccinated Aboriginal and Torres Strait Island children in north Queensland.
Methodology: A cross-sectional survey of immunity in a sample of children; 101 fully vaccinated Aboriginal and Torres Strait Island children, with a median age of 24.5 months, from 10 communities in North Queensland participated in this study. The main outcome measures were the prevalence of adequate antibody levels against hepatitis B, poliomyelitis and measles.
Results: Only 54% (95% Cl 44–63%) of the children had adequate immunity (10 m iu/mL) to hepatitis B, and one child had been infected despite vaccination. Although all the children (95% Cl 96–100%) had adequate immunity (i.e. neutralizing antibodies at a dilution of 1:8) to poliovirus 2, only 93% (95% Cl 86–96%) and 60% (95% Cl 50–69%) had adequate immunity to polioviruses 1 and 3, respectively. Nearly all (96%; 95% Cl 90–98%) of the children had adequate immunity (i.e. detectable IgG antibody) to measles.
Conclusions: Although a relatively low proportion of the children had adequate antibody levels against hepatitis B the clinical significance of this observation is uncertain. Further studies are needed to determine whether fully vaccinated Torres Strait Island children have been adequately protected and whether they require a booster dose of hepatitis B vaccine. A substantial proportion of fully vaccinated Aboriginal and Torres Strait Island children are inadequately protected against poliomyelitis, and therefore any such child with acute flaccid paralysis should be investigated fully for poliomyelitis. Vaccinated Aboriginal and Torres Strait Island children are well protected against measles, as are other Australian children. 相似文献
Methodology: A cross-sectional survey of immunity in a sample of children; 101 fully vaccinated Aboriginal and Torres Strait Island children, with a median age of 24.5 months, from 10 communities in North Queensland participated in this study. The main outcome measures were the prevalence of adequate antibody levels against hepatitis B, poliomyelitis and measles.
Results: Only 54% (95% Cl 44–63%) of the children had adequate immunity (10 m iu/mL) to hepatitis B, and one child had been infected despite vaccination. Although all the children (95% Cl 96–100%) had adequate immunity (i.e. neutralizing antibodies at a dilution of 1:8) to poliovirus 2, only 93% (95% Cl 86–96%) and 60% (95% Cl 50–69%) had adequate immunity to polioviruses 1 and 3, respectively. Nearly all (96%; 95% Cl 90–98%) of the children had adequate immunity (i.e. detectable IgG antibody) to measles.
Conclusions: Although a relatively low proportion of the children had adequate antibody levels against hepatitis B the clinical significance of this observation is uncertain. Further studies are needed to determine whether fully vaccinated Torres Strait Island children have been adequately protected and whether they require a booster dose of hepatitis B vaccine. A substantial proportion of fully vaccinated Aboriginal and Torres Strait Island children are inadequately protected against poliomyelitis, and therefore any such child with acute flaccid paralysis should be investigated fully for poliomyelitis. Vaccinated Aboriginal and Torres Strait Island children are well protected against measles, as are other Australian children. 相似文献
38.
SL Stuckey EJ Gilford PJ Smith M Kean 《Journal of Medical Imaging and Radiation Oncology》1995,39(4):350-355
This preliminary study was designed to investigate the ability of multiple axial volume three-dimensional fourier transform (3DFT) time-of-flight (TOF) magnetic resonance angiography (MRA) to depict the carotid bifurcation in the early post-carotid endarterectomy period. Five patients underwent intra-operative digital subtraction angiography (DSA) and carotid MRA within 5 days of carotid endarterectomy. An axial volume fast imaging in steady-state precession (FISP) gradient-echo 3DFT TOF carotid MRA technique in this limited series appeared to display accurately the surgically significant abnormalities at the carotid bifurcation after endarterectomy. However, in normal or near-normal intra-operative DSA studies, overestimation of internal carotid artery stenoses was encountered. Postoperative MRA demonstrates potential as a useful non-invasive investigation after carotid endarterectomy but should be interpreted with caution until larger studies become available. 相似文献
39.
Stef PJ Kremers Gert-Jan de Bruijn Tommy LS Visscher Willem van Mechelen Nanne K de Vries Johannes Brug 《The international journal of behavioral nutrition and physical activity》2006,3(1):9
Background
Studies on the impact of the 'obesogenic' environment have often used non-theoretical approaches. In this journal's debate and in other papers authors have argued the necessity of formulating conceptual models for differentiating the causal role of environmental influences on behavior. 相似文献40.
Long-term results of the lateral tunnel Fontan operation 总被引:11,自引:0,他引:11
Stamm C Friehs I Mayer JE Zurakowski D Triedman JK Moran AM Walsh EP Lock JE Jonas RA Del Nido PJ 《The Journal of thoracic and cardiovascular surgery》2001,121(1):28-41
OBJECTIVES: Completion of a total cavopulmonary anastomosis with an intra-atrial lateral tunnel is known to yield good early and midterm results. In this study, we sought to determine the long-term outcome (10 years) after a lateral tunnel Fontan procedure. METHODS: Between October 1987 and December 1991, 220 patients (aged 11 months to 32 years) with a wide range of underlying diagnoses underwent a fenestrated or nonfenestrated lateral tunnel Fontan procedure at our institution. Current follow-up information was available for 196 patients (94%, mean follow-up = 10.2 +/- 0.6 years). Risk factor analysis included patient-related and procedure-related variables, with death, failure, and bradyarrhythmia or tachyarrhythmia as outcome parameters. RESULTS: There were 12 early deaths (<30 days or hospital death), 7 late deaths, 4 successful takedown operations, and 4 heart transplantations. Kaplan-Meier estimated survival was 93% at 5 years and 91% at 10 years, and freedom from failure was 90% at 5 years and 87% at 10 years. Freedom from new supraventricular tachyarrhythmia was 96% at 5 years and 91% at 10 years; freedom from new bradyarrhythmia was 88% at 5 years and 79% at 10 years. Three patients had evidence of protein-losing enteropathy. Multivariable risk factors for development of supraventricular tachyarrhythmia included heterotaxy syndrome, atrioventricular valve abnormalities, and preoperative bradyarrhythmia. Risk factors for bradyarrhythmia included systemic venous anomalies. The sole risk factor for late failure was a previous coarctation repair. CONCLUSION: The lateral tunnel Fontan procedure results in excellent long-term outcome even when used in patients with diverse anatomic diagnoses. The incidence of atrial tachyarrhythmia is low and mainly depends on the underlying cardiac morphology and preoperative arrhythmia. The good long-term outcome after an intracardiac lateral tunnel Fontan procedure should serve as a basis for comparison with other surgical alternatives. 相似文献