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Compound heterozygosity of novel missense mutations in the gamma-glutamyl-carboxylase gene causes hereditary combined vitamin K-dependent coagulation factor deficiency 下载免费PDF全文
Darghouth D Hallgren KW Shtofman RL Mrad A Gharbi Y Maherzi A Kastally R LeRicousse S Berkner KL Rosa JP 《Blood》2006,108(6):1925-1931
Hereditary combined vitamin K-dependent (VKD) coagulation factor deficiency is an autosomal recessive bleeding disorder associated with defects in either the gamma-carboxylase, which carboxylates VKD proteins to render them active, or the vitamin K epoxide reductase (VKORC1), which supplies the reduced vitamin K cofactor required for carboxylation. Such deficiencies are rare, and we report the fourth case resulting from mutations in the carboxylase gene, identified in a Tunisian girl who exhibited impaired function in hemostatic VKD factors that was not restored by vitamin K administration. Sequence analysis of the proposita did not identify any mutations in the VKORC1 gene but, remarkably, revealed 3 heterozygous mutations in the carboxylase gene that caused the substitutions Asp31Asn, Trp157Arg, and Thr591Lys. None of these mutations have previously been reported. Family analysis showed that Asp31Asn and Thr591Lys were coallelic and maternally transmitted while Trp157Arg was transmitted by the father, and a genomic screen of 100 healthy individuals ruled out frequent polymorphisms. Mutational analysis indicated wild-type activity for the Asp31Asn carboxylase. In contrast, the respective Trp157Arg and Thr591Lys activities were 8% and 0% that of wild-type carboxylase, and their compound heterozygosity can therefore account for functional VKD factor deficiency. The implications for carboxylase mechanism are discussed. 相似文献
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Lenarsky C; Weinberg K; Guinan E; Dukes PP; Barak Y; Ortega J; Siegel S; Williams K; Lazerson J; Weinstein H 《Blood》1988,71(1):226-229
Constitutional pure red cell aplasia (CPRCA) is a syndrome of failed erythropoiesis usually diagnosed within the first year of life. Four patients with CPRCA received transplants with marrow from their HLA- identical, mixed lymphocyte culture-nonreactive siblings. All patients were resistant to corticosteroid therapy and were dependent on regular red cell transfusions for at least 5 years. Three patients were conditioned with procarbazine, antithymocyte globulin, cyclophosphamide, and busulfan, and one was conditioned with antithymocyte serum, cyclophosphamide, and busulfan. Three patients promptly had successful engraftments with establishment of donor hematopoiesis. One patient initially rejected his graft but received a successful retransplant. All patients are currently alive with Karnofsky performance scores of 100 and normal erythropoiesis of donor origin. Despite a history of multiple transfusions, bone marrow transplantation is a potentially curative therapy for patients with CPRCA. 相似文献
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Rima Moghnieh Dania Abdallah Lyn Awad Tamima Jisr Anas Mugharbil Ali Youssef Hani Tamim Samer Khaldieh Oula Massri Najat Rashini Youssef Hamdan Ahmad Ibrahim 《Infection》2018,46(6):823-835
Purpose
In this study, we assessed the incidence, contributing factors and outcome of prolonged neutropenia above 7 days and of bacteraemia in patients with lymphoma and multiple myeloma who underwent autologous haematopoietic stem cell transplantation (AHSCT) without antibacterial prophylaxis.Methods
This is a retrospective chart review of 190 adult patients who underwent AHSCT between 2005 and 2015 at a Lebanese hospital.Results
Neutropenia of 7 days duration and longer was documented in 66% of the patient population. Through univariate analysis, patients with lymphoma were significantly more likely to have prolonged neutropenia (≥?7 days) compared to those with myeloma. Mucositis above grade 3, diarrhoea and fever were more likely to occur in patients with prolonged neutropenia. Bacteraemia was documented in 12.6% of the patients. Total mortality rate was 3.7%, and that attributed to bacteraemia was 12.5% in the bacteraemia subgroup. Among bacterial isolates recovered from clinical specimens (89 isolates), 70% were Gram-negative, of which 57% were fluoroquinolone susceptible. Ninety-five percent of the Gram-negative bacteria causing bacteraemia were susceptible to fluoroquinolones.Conclusion
Bacterial pathogens causing bacteraemia were still highly susceptible to fluoroquinolones, despite the high prevalence of fluoroquinolone-resistant strains in the general bacterial ecology. Accordingly, the pertinence of fluoroquinolone prophylaxis in the AHSCT setting warrants further investigation. Moreover, continuous surveillance of local antibiograms in this patient population has become a must in an era of preponderant antibiotic resistance.37.
Nihal Fathi Samar H. Goma Nadia M. Ismail Abeer M. Ghandour Sally S. Youssef Nisreen A. Mohammed Eman Mosad 《The Egyptian Rheumatologist》2018,40(1):45-49
Background
Systemic sclerosis (SSc) is an autoimmune connective tissue disease with vascular, fibrotic and immune changes of skin and some internal organs. Anti-heterogeneous nuclear ribonucleoproteins (anti-hnRNP) were found in SSc patients.Aim of the work
To assess anti-hnRNP A1 and A2 autoantibodies in limited SSc patients and to find their relation to clinical and hand radiographic characteristics.Patients and methods
26 limited SSc patients and 16 matched control were studied. Skin thickness was scored according to the modified Rodnan skin score method (mRss) and radiologic examination by plain X-ray of the hand and wrist was performed anti-hnRNP A1 and A2 were measured in patients and control.Results
All patients were females with a mean age of 37.5 ± 11.24 years and mean disease duration of 7.84 ± 1.19 years. 96.2% of cases showed juxta-articular osteoporosis, 38.5% with marginal erosions, 73.1% with surface erosions, 42.3% with subchondral cyst, 42.3% with metacarpophalangeal subluxation, 11.5% with marginal sclerosis, 80.8% with resorption of distal phalanges, 38.5% with resorption of distal ulna and 34.6% with calcinosis. Anti-hnRNPA1 was positive in all the patients but the anti-hnRNPA2 was positive in 21 (80.8%). Anti-hnRNP A1 and A2 showed significant difference between patients and control (5.66 ± 4.18 ng/ml vs 2.88 ± 0.82; p < 0.01 and 1.82 ± 0.36 vs 0.73 ± 0.08; p < 0.02, respectively). There was no significant correlation between the markers with the mRss or radiographic changes.Conclusion
Joint affection in SSc is more frequent than expected. Anti-hnRNP A1 and anti hnRNP A2 antigens may be useful markers for SSc patient although no significant relation was found with radiologic findings. 相似文献38.
Holschermann H; Durfeld F; Maus U; Bierhaus A; Heidinger K; Lohmeyer J; Nawroth PP; Tillmanns H; Haberbosch W 《Blood》1996,88(10):3837-3845
39.
Carrier detection in hemophilia A: a cooperative international study. I. The carrier phenotype 总被引:1,自引:0,他引:1
Graham JB; Rizza CR; Chediak J; Mannucci PM; Briet E; Ljung R; Kasper CK; Essien EM; Green PP 《Blood》1986,67(6):1554-1559
Eight laboratories in six countries cooperated to clarify several issues concerning the phenotypes of heterozygous carriers of hemophilia "A." Plasma levels of factor VIII (F.VIII:C, formerly VIII:C) and von Willebrand factor (VWF:Ag, formerly VIIIR:Ag) of carriers and normal women were determined by various "in-house" methods; a single lyophilized plasma standard was used for all assays. Analysis of the collated data from 336 carriers (296 obligatory carriers and 40 sporadic carriers) and 137 normal women showed that there was no difference in the F.VIII:C levels of "paternal" carriers (women who had obtained the abnormal gene from their fathers) and "maternal" carriers. Neither was there a difference in the VWF:Ag levels of normal women and either type of carrier. Age was found to have a significant effect on both F.VIII:C and VWF:Ag, values being higher at very young and very old ages, the minima occurring in the 25- to 30-year range. ABO blood type had a striking effect. Women of types A, B, and AB (designated non- O in the study), both normals and carriers, had significantly higher levels of both factors than did women of type O. Analysis by laboratories showed that differences in mean levels of both factors between laboratories were highly significant. It was concluded that age, ABO blood type, and laboratory variation should be taken into account in carrier detection. 相似文献
40.