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651.
Abdeljalil Elgot Samir Ahboucha My. Mustapha Bouyatas Michlle Fvre-Montange Halima Gamrani 《Neuroscience letters》2009,466(1):6-10
Water deprivation is a stress that has been associated with activation of several endocrine systems, including circumventricular organs of the central nervous system. The sub-comissural organ (SCO), characterized by its glycoprotein secretion called Reissner's fiber has been suggested to play a role in the regulation of body water balance. Meriones shawi, a semi-desertic rodent characterized by its resistance to long periods of thirst was subjected to water deprivation for 1 and 3 months. Effect of water deprivation was evaluated immunohistochemically on 5-hydroxytryptamine (5-HT; serotonin) system and glycoprotein secretion of the SCO. Our findings demonstrate significant reduction of anti-Reissner's fiber immunoreactive materials within basal and apical parts of the SCO ependymocytes. These changes seem to be the consequence of reduced control by 5-HT fibers reaching the SCO as a concomitant and significant reduction of anti-5-HT immunoreactive fibers are also observed following water deprivation. 5-HT immunoreactive reduction is seen in several regions in the brain including the neurons of origin within the dorsal raphe nucleus and the projecting supra and sub-ependymal fibers reaching the classical ependyma of the third ventricle. The extent of Reissner's fiber and 5-HT immunoreactive changes significantly correlates with the severity of water restriction. We suggest that water deprivation causes changes of the classical ependyma and the specialized ependyma that differentiates into the SCO as well as other cirumventricular organs such as the subfornical organ and the organum vasculosum laminae terminalis known to control drinking behaviors. 相似文献
652.
653.
Bourcier R Papagiannaki C Bibi R Cottier JP Herbreteau D 《Clinical neurology and neurosurgery》2012,114(9):1248-1253
Objective
The objective of this communication was to study the incidence and course of ICD and the long term outcome of this severe disease. The second goal was to analyze the different endovascular treatment modalities according to their long term results.Methods
It is a retrospective analysis of 14 patients with ICD admitted in a single center in two consecutive years, treated with endovascular procedures. Patients harbouring blister-like ruptured intracranial aneurysms were excluded from this study.Results
In this case series, 12 patients presented with subarachnoid hemorrhage (SAH) and 2 with brain stem symptoms. Mean age was 51 years and 13 patients were female. Six patients (43%) died and 8 (57%) survived with a mRS at 0–1. Mean follow up was 21 months.Conclusions
ICD as a cause of SAH seems to be more frequent than previously thought and usually has a severe course. It requires a high level of suspicion to diagnosis and specific endovascular treatment modalities are required for each location to ensure long term stability and change the poor prognosis. 相似文献654.
Aluminum (Al) is a neurotoxic agent that accumulates in the substantia nigra of patients affected by Parkinson's disease and in other cerebral areas of different neurodegenerative pathologies. Al has been associated with neuronal and glial dysfunctions, and neuronal changes have been suggested to affect several neurotransmitter systems including the dopaminergic system. The present study was designed to evaluate by means of immunohistochemistry using antibodies against tyrosine hydroxylase (TH; the rate-limiting enzyme of dopamine synthesis) the effects of chronic Al exposure (0, 3%) in drinking water during 4 months in adulthood or since intra-uterine age in the substantia nigra. Our results show a significant decrease in the number of cells labeled by the antibody against TH in rats treated with Al compared to controls. The TH-immunoreactive decrease following Al treatment is accentuated in the rat group treated since intrauterine age. In both treated groups, Al exposure induced a significant decrease of locomotor performance. Interestingly, as for TH-immunoreactivity, the decreased locomotor activity was also accentuated in the group treated since intrauterine age. The Al-induced TH alterations may be one of the causes of aluminum-induced neurotoxicity. 相似文献
655.
656.
Ben-Shem A Fass D Bibi E 《Proceedings of the National Academy of Sciences of the United States of America》2007,104(2):462-466
Intramembrane proteases catalyze peptide bond cleavage of integral membrane protein substrates. This activity is crucial for many biological and pathological processes. Rhomboids are evolutionarily widespread intramembrane serine proteases. Here, we present the 2.3-A-resolution crystal structure of a rhomboid from Escherichia coli. The enzyme has six transmembrane helices, five of which surround a short TM4, which starts deep within the membrane at the catalytic serine residue. Thus, the catalytic serine is in an externally exposed cavity, which provides a hydrophilic environment for proteolysis. Our results reveal a mechanism to enable water-dependent catalysis at the depth of the hydrophobic milieu of the membrane and suggest how substrates gain access to the sequestered rhomboid active site. 相似文献
657.
We here present the first report of the detection of two rare beta0-thalassemia (thal) mutations in the Tunisian population: codon 47 (+A) and codons 106/107 (+G). To the best of our knowledge this is the second report of the codon 47 (+A) mutation, the first being identified in a Surinamese subject. The codons 106/107 (+G) mutation was first described in American Blacks, subsequently in Egyptians and Palestinians, and now in Tunisians. These mutations were detected by denaturing gradient gel electrophoresis (DGGE) screening followed by automated nucleotide sequencing. The former was found in two related beta-thal major patients in the homozygous state, while the latter was identified in a homozygous state in a transfusion-dependent beta-thal subject and in a sickle cell beta-thal patient. Both mutations are in linkage disequilibrium with haplotype V and sequence framework 2. Given the known wide spectrum of beta-thal alleles in the Tunisian population, the present report further confirms such heterogeneity. The knowledge of an updated spectrum of beta-thal alleles in Tunisia must allow the implementation of a more efficient screening strategy for genetic counseling and prenatal diagnosis. 相似文献
658.
The polymorphism of the beta-globin gene haplotypes and frameworks are useful in the determination of the unicentric and multicentric origin of a mutational event. In order to improve our knowledge of the chromosomal background of the beta-globin gene in three beta-thalassemia (thal) mutations originally reported in Tunisia, namely codons 25/26 (+T), codon 30 (G-->C) and IVS-I-2 (T-->G), we have investigated 13 unrelated individuals. There were five non transfusion-dependent patients homozygous for the IVS-I-2 (T-->G) mutation, five others were homozygous for the codon 30 (G-->C) mutation, one was a homozygote for the codons 25/26 (+T) insertion mutation and one patient was a compound heterozygote for the codon 39 (C-->T) and codon 25/26 (+T) mutations; the last patient had a betaS/codon 25/26 (+T) compound heterozygous genotype. Haplotype analysis was carried out by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) based methods. The framework polymorphism was established by direct sequencing. beta-Globin gene analyses demonstrated that all IVS-I-2 (T-->G) cases were associated with haplotype IX; the codon 30 (G-->C) mutation was supported by haplotype I, while the codons 25/26 (+T) mutation was linked to haplotypes I and IX. 相似文献
659.
Abidi O Boulouiz R Nahili H Ridal M Alami MN Tlili A Rouba H Masmoudi S Chafik A Hassar M Barakat A 《International journal of pediatric otorhinolaryngology》2007,71(8):1239-1245
OBJECTIVE: Mutations in the connexin 26 gene (GJB2), which encodes a gap-junction protein expressed in the inner ear, have been shown to be responsible for a major part of autosomal recessive non-syndromic hearing loss in Caucasians. The aim of our study was to determine the prevalence and spectrum of GJB2 mutations, including the (GJB6-D13S1830) deletion, in Moroccan patients and estimate the carrier frequency of the 35delG mutation in the general population. METHODS: Genomic DNA was isolated from 81 unrelated Moroccan familial cases with moderate to profound autosomal recessive non-syndromic hearing loss and 113 Moroccan control individuals. Molecular studies were performed using PCR-Mediated Site Directed Mutagenesis assay, PCR and direct sequencing to screen for GJB2, 35delG and del(GJB6-D13S1830) mutations. RESULTS: GJB2 mutations were found in 43.20% of the deaf patients. Among these patients 35.80% were 35delG/35delG homozygous, 2.47% were 35delG/wt heterozygous, 3.70% were V37I/wt heterozygous, and 1 patient was E47X/35delG compound heterozygous. None of the patients with one or no GJB2 mutation displayed the common (GJB6-D13S1830) deletion. We found also that the carrier frequency of GJB2-35delG in the normal Moroccan population is 2.65%. CONCLUSIONS: These findings indicate that the GJB2-35delG mutation is the major cause of autosomal recessive non-syndromic hearing loss in Moroccan population. Two other mutations were also detected (V37I and E47X), in agreement with similar studies in other populations showing heterogeneity in the frequencies and types of mutation in connexin 26 gene. 相似文献
660.
Danielle Claude Bilanda Théophile Dimo Paul Désiré Dzeufiet Djomeni Ndzana Martin Thierry Bella Oumarou Bibi Farouck Aboubakar Télesphore Benoit Nguelefack Paul V. Tan Pierre Kamtchouing 《Journal of ethnopharmacology》2010