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91.
92.
Rie Anzai Megumi Tsuji Sumimasa Yamashita Yoshinao Wada Nobuhiko Okamoto Hirotomo Saitsu Naomichi Matsumoto Tomohide Goto 《Brain & development》2021,43(3):402-410
AimMOGS mutations cause congenital disorders of glycosylation type IIb (CDG-IIb or GCS1-CDG). The specific manifestations caused by the mutations in this gene remain unknown. We aimed to describe the clinical features of CDG- IIb and the effectiveness of urinary oligosaccharide analysis in the diagnosis of CDG- IIb.MethodsPatient 1 was analyzed with whole-exome sequencing (WES) to identify the causative gene of intractable epilepsy and severe developmental delay. After detecting MOGS mutation in patient 1, we analyzed patients 2 and 3 who were siblings and had clinical features similar to those in patient 1. Urinary oligosaccharide analysis was performed to confirm CDG- IIb diagnosis in patient 1. The clinical features of these patients were analyzed and compared with those in eight published cases.ResultsOur three patients presented with early infantile epileptic encephalopathy, generalized hypotonia, hepatic dysfunction and dysmorphic features. In two cases, compound heterozygous mutations in MOGS were identified by WES. Isolation and characterization of the urinary oligosaccharide was performed in one of these cases to confirm the diagnosis of CDG-IIb. Although the isoelectric focusing of transferrin (IEF-T) of serum in this patient was normal, urinary excretion of Hex4 corresponding to Glc3Man was observed by mass spectrometry.ConclusionThis report provides clinical manifestations of CDG-IIb with MOGS mutation. CDG-IIb shows a normal IEF profile of serum transferrin and cannot be detected by structural analysis of the patient’s glycoproteins. Characterization of urinary oligosaccharides should be considered to detect this disorder. 相似文献
93.
Daisuke Tajima Takuji Nakamura Fumio Ichinose Nobuhiko Okamoto Yuko Tomonoh Keiko Uda Rie Furukawa Katsuya Tashiro Muneaki Matsuo 《Brain & development》2021,43(3):482-485
Paroxysmal abnormal eye movement in early infancy is one of the initial symptoms of glucose transporter 1 deficiency syndrome (GLUT1DS). We describe four early infants with transient hypoglycorrhachia presenting with abnormal eye movements. Their symptoms disappeared after the introduction of a ketogenic diet (KD), and their development was normal. Since no variants in SLC2A1 were detected, the CSF-to-blood glucose ratios (C/B) were re-examined, and within normal range. None of the four patients displayed recurrent symptoms after withdrawal from the KD. Because long-term KD has potential adverse effects and could affect the quality of life of patients and their families, re-examination of CSF glucose during late infancy should be considered in the case of absence of the SLC2A1 pathogenic variant. 相似文献
94.
Masayuki Nakajima Masashi Matsuyama Naoki Arai Hideyasu Yamada Kentaro Hyodo Mizu Nonaka Haruna Kitazawa Kazufumi Yoshida Rie Shigemasa Yuko Morishima Takumi Kiwamoto Hironori Masuko Yukio Ishii Masafumi Muratani Takefumi Saito Nobuyuki Hizawa 《The Journal of allergy and clinical immunology》2021,147(2):772-775
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97.
Okumura Y Watanabe I Oshikawa N Masaki R Okubo K Hashimoto K Kofune T Yamada T Wakita R Takagi Y Saito S Ozawa Y Kanmatsuse K 《Japanese heart journal》2003,44(5):655-666
Atrioventricular nodal reentrant tachycardia (AVNRT) is a relatively common paroxysmal supraventricular tachycardia. This study investigated whether adenosine-5'-triphosphate (ATP) injection during sinus rhythm might be useful in the noninvasive diagnosis of dual AV nodal pathways. The study group consisted of 9 patients with slow/fast AVNRT and 11 control patients without antegrade dual AV nodal physiology (DAVNP). ATP (2.5 to 30 mg, in 2.5-mg increments was injected during sinus rhythm until signs of DAVNP (> or = 50 msec increase or decrease in AH or PR interval in two consecutive beats) or > or = second-degree AV block was observed. DAVNP was diagnosed by ATP test in all 9 patients with slow/fast AVNRT. DAVNP was observed by ATP test in 3 of the 11 control patients. Thus, the test had a sensitivity of 100% and specificity of 73%. ATP test given during sinus rhythm is useful for identifying patients with dual AV nodal pathways who are prone to AVNRT. 相似文献
98.
J Azuma A Sawamura H Harada T Tanimoto T Ishiyama Y Morita Y Yamamura N Sperelakis 《Journal of molecular and cellular cardiology》1981,13(6):577-587
The catecholamines exert a positive inotropic effect associated with elevated tissue cyclic AMP levels and possibly with increase in the number of membrane slow cationic channels available for voltage activation. In the present study, catecholamines (isoproterenol, dopamine and dobutamine) were tested for their ability to affect the maximum upstroke velocity (+ Vmax) of the slow action potentials, the first derivative () of developed tension accompanying the slow responses, and the tissue cyclic AMP levels in the ventricular myocardium of isolated perfused chick hearts. To study the slow channels exclusively, the fast Na+ channels were voltage inactivated by elevated (25 mm) K+. In this condition of functional removal of the fast channels, the heart could not be excited by intense electrical stimulation. It was found that these catecholamines induced slow action potentials accompanied by contractions. Elevation of the concentration of these agents produced increases in + Vmax, , and cyclic AMP in a dose-dependent fashion; a close correlation was obtained between the cyclic AMP level, + Vmax and . These results support the hypothesis that the increases in + Vmax of the slow action potentials and in contraction are explained by increase in the number of available slow channels mediated by intracellular cyclic AMP levels, and the resulting increase in the Ca2+ influx. 相似文献
99.
Resistance to the Bacillus thuringiensis bioinsecticide in a field population of Plutella xylostella is due to a change in a midgut membrane receptor. 总被引:21,自引:3,他引:21
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J Ferré M D Real J Van Rie S Jansens M Peferoen 《Proceedings of the National Academy of Sciences of the United States of America》1991,88(12):5119-5123
The biochemical mechanism for resistance to Bacillus thuringiensis crystal proteins was studied in a field population of diamondback moths (Plutella xylostella) with a reduced susceptibility to the bioinsecticidal spray. The toxicity and binding characteristics of three crystal proteins [CryIA(b), CryIB, and CryIC] were compared between the field population and a laboratory strain. The field population proved resistant (greater than 200-fold compared with the laboratory strain) to CryIA(b), one of the crystal proteins in the insecticidal formulation. Binding studies showed that the two strains differ in a membrane receptor that recognizes CryIA(b). This crystal protein did not bind to the brush-border membrane of the midgut epithelial cells of the field population, either because of strongly reduced binding affinity or because of the complete absence of the receptor molecule. Both strains proved fully susceptible to the CryIB and CryIC crystal proteins, which were not present in the B. thuringiensis formulation used in the field. Characteristics of CryIB and CryIC binding to brush-border membranes of midgut epithelial cells were virtually identical in the laboratory and the field population. 相似文献
100.
Aisa Y Mori T Nakazato T Yamazaki R Yamagami J Amagai M Ikeda Y Okamoto S 《International journal of hematology》2005,82(3):266-269
A 44-year-old woman with refractory follicular lymphoma underwent allogeneic stem cell transplantation (SCT) and achieved
complete remission. Grade III acute graft-versus-host disease (GVHD) developed on day 23, but no chronic GVHD occurred. The
patient developed severe erosion with bullous lesions in the oral cavity 18 months after SCT. At that time, the lymphoma remained
in complete remission, and she had no clinical or laboratory findings suggesting chronic GVHD. A biopsy of the oral mucosa
showed moderate lymphoplasmacytic infiltration and subepidermal bullae, and direct immunofluorescence staining demonstrated
linear deposition of C3 at the dermo-epidermal junction. An immunoblotting assay using human epidermal extracts confirmed
the presence in her serum of an antibody against the 230-kd bullous pemphigoid antigen 1 (BPAG1). A diagnosis of cicatricial
pemphigoid (CP) was made, and complete resolution of the CP was achieved with pred-nisolone therapy. The occurrence of autoimmune
blistering diseases is rare after allogeneic SCT. 相似文献