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排序方式: 共有887条查询结果,搜索用时 62 毫秒
1.
D. Gareth Evans Kelly-Anne Phillips Roger L. Milne Robert Fruscio Cezary Cybulski Jacek Gronwald Jan Lubinski Tomasz Huzarski Zerin Hyder Claire Forde Kelly Metcalfe Leigha Senter Jeffrey Weitzel Nadine Tung Dana Zakalik Maria Ekholm Ping Sun Steven A. Narod kConFab Investigators Polish Hereditary
Breast Cancer Consortium Hereditary Breast Cancer Clinical Study Group 《British journal of cancer》2021,124(9):1524
Background The impact of various breast-cancer treatments on patients with a BRCA2 mutation has not been studied. We sought to estimate the impact of bilateral oophorectomy and other treatments on breast cancer-specific survival among patients with a germline BRCA2 mutation.Methods We identified 664 women with stage I–III breast cancer and a BRCA2 mutation by combining five different datasets (retrospective and prospective). Subjects were followed for 7.2 years from diagnosis to death from breast cancer. Tumour characteristics and cancer treatments were patient-reported and derived from medical records. Predictors of survival were determined using Cox proportional hazard models, adjusted for other treatments and for prognostic features.Results The 10-year breast-cancer survival for ER-positive patients was 78.9% and for ER-negative patients was 82.3% (adjusted HR = 1.23 (95% CI, 0.62–2.45, p = 0.55)). The 10-year breast-cancer survival for women who had a bilateral oophorectomy was 89.1% and for women who did not have an oophorectomy was 59.0% (adjusted HR = 0.45; 95% CI, 0.28–0.72, p = 0.001). The adjusted hazard ratio for chemotherapy was 0.83 (95% CI, 0.65–1.53: p = 0.56).Conclusions For women with breast cancer and a germline BRCA2 mutation, positive ER status does not predict superior survival. Oophorectomy is associated with a reduced risk of death from breast cancer and should be considered in the treatment plan.Subject terms: Targeted therapies, Breast cancer 相似文献
2.
M. Szymczak P. Kaliciński G. Kowalewski M. Markiewicz-Kijewska D. Broniszczak H. Ismail M. Stefanowicz A. Kowalski J. Rubik I. Jankowska B. Piątosa J. Teisseyre R. Grenda 《Transplantation proceedings》2018,50(7):2140-2144
Combined liver-kidney transplantation (CLKT) is a rare procedure in pediatric patients in which liver and kidney from 1 donor are transplanted to a recipient during a single operation. The aim of our study was to analyze indications and results of CLKT in children.
Materials and Methods
Between 1990 and 2017 we performed 722 liver transplantations in children; we performed 920 kidney transplantations in children since 1984. Among them, 25 received CLKT. Primary diagnosis was fibro-polycystic liver and kidney disease in 17 patients, primary hyperoxaluria type 1 in 6 patients, and atypical hemolytic uremic syndrome-related renal failure in 2 children. Age of patients at CLKT was 3 to 23 years (median 16 years) and body mass was 11 to 55 kg (median 35.5kg). All patients received whole liver graft. Kidney graft was transplanted after liver reperfusion before biliary anastomosis. Cold ischemia time was 5.5 to 13.3 hours (median 9.4 hours) for liver transplants and 7.3 to 15 hours (median 10.4 hours) for kidney transplants. In 8 patients X-match was positive. We analyzed posttransplant (Tx) course and late results in our group of pediatric recipients of combined grafts.Results
Tx follow-up ranged from 1.5 to 17 years (median 4.5 years). Two patients died: 1 patient with oxalosis lost renal graft and died 2.6 years after Tx due to complications of long-term dialysis, and 1 died due to massive bleeding in early postoperative period. Twelve patients were transferred under the care of adult transplantation centers. Six patients were dialyzed after CLKT due to acute tubular necrosis, and time of kidney function recovery was 10 to 27 days in these patients. In 1 patient with aHUS, renal function did not recover. In children with oxalosis, hemodialysis was performed for 1 month after Tx as a standard, with the aim to remove accumulated oxalate. Primary immunosuppression consisted of daclizumab or basiliximab, tacrolimus, mycophenolate mofetil, and steroids. Acute rejection occurred in 4 liver and 3 kidney grafts. One patient required liver retransplantation due to hepatitis C virus recurrence and 2 patients required kidney retransplantation. Two patients required dialysis.Conclusions
CLKT in children results in low rate of rejection and high rate of patient and graft survival. 相似文献3.
Cezary Wójcik Sergio Fazio Adam D. McIntyre Robert A. Hegele 《Journal of clinical lipidology》2018,12(5):1146-1150
We describe a case of a 36-year-old woman with severe hypertriglyceridemia likely caused by double heterozygosity of a known pathogenic APOA5 nonsense variant (p.Q275X) and a novel CREB3L3 nonsense variant (p.C296X) on a background of very strong polygenic susceptibility. Her clinical course worsened with development of eruptive xanthomata after oral administration of 2 mg estradiol twice daily for 2 weeks as part of a medical protocol for intrauterine embryo transfer following in vitro fertilization. Her triglyceride levels decreased to baseline and xanthomata resolved without treatment after discontinuation of hormonal therapy, which also resulted in termination of pregnancy. Before undergoing a second embryo transfer using her natural cycle and no exogenous hormones, the patient started combination therapy with eicosapentaenoic acid ethyl ester and gemfibrozil, leading to an ~80% decrease in triglyceride levels. She continued treatment throughout pregnancy, which progressed to term with the delivery of healthy twins. 相似文献
4.
5.
Factors influencing ovulation and the risk of ovarian cancer in BRCA1 and BRCA2 mutation carriers 下载免费PDF全文
Joanne Kotsopoulos Jan Lubinski Jacek Gronwald Cezary Cybulski Rochelle Demsky Susan L. Neuhausen Charmaine Kim‐Sing Nadine Tung Susan Friedman Leigha Senter Jeffrey Weitzel Beth Karlan Pal Moller Ping Sun Steven A. Narod 《International journal of cancer. Journal international du cancer》2015,137(5):1136-1146
The role of the lifetime number of ovulatory cycles has not been evaluated in the context of BRCA‐associated ovarian cancer. Thus, we conducted a matched case–control study to evaluate the relationship between the cumulative number of ovulatory cycles (and contributing components) and risk of developing ovarian cancer in BRCA mutation carriers (1,329 cases and 5,267 controls). Information regarding reproductive and hormonal factors was collected from a routinely administered questionnaire. Conditional logistic regression was used to evaluate all associations. We observed a 45% reduction in the risk of developing ovarian cancer among women in the lowest vs. highest quartile of ovulatory cycles (OR = 0.55; 95% CI 0.41–0.75, p = 0.0001). Breastfeeding for more than 12 months was associated with a 38% (95% CI 0.48–0.79) and 50% (95% CI 0.29–0.84) reduction in risk among BRCA1 and BRCA2 mutation carriers, respectively. For oral contraceptive use, maximum benefit was seen with five or more years of use among BRCA1 mutation carriers (OR = 0.50; 95% CI 0.40–0.63) and three or more years for BRCA2 mutation carriers (OR = 0.42; 95% CI 0.22–0.83). Increasing parity was associated with a significant inverse trend among BRCA1 (OR = 0.87; 95% CI 0.79–0.96; p‐trend = 0.005) but not BRCA2 mutation carriers (OR 0.98; 95% CI 0.81–1.19; p‐trend = 0.85). A later age at menopause was associated with an increased risk in women with a BRCA1 mutation (OR trend = 1.18; 95% CI 1.03–1.35; p = 0.02). These findings support an important role of breastfeeding and oral contraceptive use for the primary prevention of ovarian cancer among women carrying BRCA mutations. 相似文献
6.
Monika Siołek Cezary Cybulski Danuta Gąsior‐Perczak Artur Kowalik Beata Kozak‐Klonowska Aldona Kowalska Małgorzata Chłopek Wojciech Kluźniak Dominika Wokołorczyk Iwona Pałyga Agnieszka Walczyk Katarzyna Lizis‐Kolus Ping Sun Jan Lubiński Steven A. Narod Stanisław Góźdż 《International journal of cancer. Journal international du cancer》2015,137(3):548-552
Mutations in the cell cycle checkpoint kinase 2 (CHEK2) tumor suppressor gene are associated with multi‐organ cancer susceptibility including cancers of the breast and prostate. A genetic association between thyroid and breast cancer has been suggested, however little is known about the determinants of this association. To characterize the association of CHEK2 mutations with thyroid cancer, we genotyped 468 unselected patients with papillary thyroid cancer and 468 (matched) cancer‐free controls for four founder mutations of CHEK2 (1100delC, IVS2 + 1G>A, del5395 and I157T). We compared the family histories reported by patients with a CHEK2 mutation to those of non‐carriers. A CHEK2 mutation was seen in 73 of 468 (15.6%) unselected patients with papillary thyroid cancer, compared to 28 of 460 (6.0%) age‐ and sex‐matched controls (OR 3.3; p < 0.0001). A truncating mutation (IVS2 + 1G>A, 1100delC or del5395) was associated with a higher risk of thyroid cancer (OR = 5.7; p = 0.006), than was the missense mutation I157T (OR = 2.8; p = 0.0001). CHEK2 mutation carriers reported a family history of breast cancer 2.2 times more commonly than non‐carriers (16.4% vs.8.1%; p = 0.05). A CHEK2 mutation was found in seven of 11 women (63%) with multiple primary cancers of the breast and thyroid (OR = 10; p = 0.0004). These results suggest that CHEK2 mutations predispose to thyroid cancer, familial aggregations of breast and thyroid cancer and to double primary cancers of the breast and thyroid. 相似文献
7.
Grzegorz Sukiennicki Magdalena Muszyńska Katarzyna Jaworska-Bieniek Katarzyna Kaczmarek Wojciech Marciniak Marcin Lener Katarzyna Durda Tomasz Gromowski Tomasz Huzarski Tomasz Byrski Jacek Gronwald Oleg Oszurek Cezary Cybulski Tadeusz Dębniak Antoni Morawski Anna Jakubowska Jan Lubiński 《Hereditary cancer in clinical practice》2015,13(Z2):A5
8.
Karol Czubak Marzena Anna Lewandowska Katarzyna Klonowska Krzysztof Roszkowski Janusz Kowalewski Marek Figlerowicz Piotr Kozlowski 《Oncotarget》2015,6(27):23399-23416
A growing body of evidence indicates that miRNAs may be a class of genetic elements that can either drive or suppress oncogenesis. In this study we analyzed the somatic copy number variation of 14 miRNA genes frequently found to be either over- or underexpressed in lung cancer, as well as two miRNA biogenesis genes, DICER1 and DROSHA, in non-small-cell lung cancer (NSCLC). Our analysis showed that most analyzed miRNA genes undergo substantial copy number alteration in lung cancer. The most frequently amplified miRNA genes include the following: miR-30d, miR-21, miR-17 and miR-155. We also showed that both DICER1 and DROSHA are frequently amplified in NSCLC. The copy number variation of DICER1 and DROSHA correlates well with their expression and survival of NSCLC and other cancer patients. The increased expression of DROSHA and DICER1 decreases and increases the survival, respectively. In conclusion, our results show that copy number variation may be an important mechanism of upregulation/downregulation of miRNAs in cancer and suggest an oncogenic role for DROSHA. 相似文献
9.
Henryk W. Witas Marcin Różalski Cezary Watala Wojciech Mlynarski RadosLAw Sychowski Jerzy Bodalski 《Platelets》2013,24(2-3):169-177
This study examined the significance of selected parameters of primary haemostasis to discriminate between relatives of children with insulin-dependent diabetes mellitus (IDDM). Platelet function, including markers of spontaneous and agonist-induced platelet activation (CD62), platelet consumption (microparticles) and clumping (aggregates), as well as selected parameters of the fibrinolytic system (t-PA and PAI-1), were studied in IDDM children (n = 45), their parents (n = 65), siblings (n = 17) and unrelated healthy controls (n = 51). The fraction of activated platelets circulating in whole blood amounted to 4.3±2.1% in IDDM children, and significantly exceeded the level found in parents (1.3±0.7%, P < 0.002), siblings (1.2±1.0%, P < 0.002), and controls (1.2±0.6%, P < 0.002). Furthermore, an enhanced formation of platelet microparticles was observed in the IDDM group, both in resting platelets and also when platelets were stimulated with thrombin. Significantly decreased total PAI-1 occurred in IDDM children (P < 0.02 versus parents); also slightly lowered active PAI-1 and t-PA antigen were noticed in IDDM subjects compared to other groups, however, the differences were not statistically significant. To assess dissimilarities between the groups of subjects we applied the forward stepwise model of discriminant function analysis, which included platelet flow cytometry parameters. The best separation and the highest discrepancy (expressed as the so called squared Mahalanobis distances, dM) was revealed between controls and IDDM patients (P ? 0.0001) and between controls and parents (P ? 0.0001). The values of dM found between IDDM children and their siblings (P < 0.001), as well as parents (P < 0.01), were of much lower significance. The finding that the control group, representing unrelated subjects, remains particularly well separated from the other groups, more or less clustered together, implies the possible involvement of genetic factor(s) which might potentially affect platelet activation and reactivity. In addition, the distinguished distribution of HLA DQAI52 and HLA DQBI57 genotypes in the groups further validates the suspicion that the altered platelet function and response in diabetes might be associated with some independent genetic factor(s), and is not likely to result from HLA DQAI52 and HLA DQBI57 impact. 相似文献
10.
The detonation gun sprayed Fe-Al type coatings as an alternative for austenitic valve steel, were investigated using two different methods of testing corrosion resistance. High temperature, 10-hour isothermal oxidation experiments at 550, 750, 950 and 1100 °C show differences in the oxidation behavior of Fe-Al type coatings under air atmosphere. The oxide layer ensures satisfying oxidation resistance, even at 950 and 1100 °C. Hematite, α-Al2O3 and metastable alumina phases were noticed on the coatings top surface, which preserves its initial thickness providing protection to the underlying substrate. In general, only negligible changes of the phase composition of the coatings were noticed with simultaneous strengthening controlled in the micro-hardness measurements, even after 10-hours of heating at 1100 °C. On the other hand, the electrochemical corrosion tests, which were carried out in 200 ppm Cl− (NaCl) and pH ~4 (H2SO4) solution to simulate the acid-rain environment, reveal higher values of the breakdown potential for D-gun sprayed Fe-Al type coatings than the ones for the bulk Fe-Al type alloy and Cr21Mn9Ni4 austenitic valve steel. This enables these materials to be used in structural and multifunctional applications in aggressive environments, including acidic ones. 相似文献