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排序方式: 共有325条查询结果,搜索用时 921 毫秒
91.
M Silengo E Belligni C Molinatto G Baldassare E Biamino N Chiesa O Zuffardi S Girirajan EE Eichler GB Ferrero 《Clinical genetics》2010,77(1):28-31
Silengo M, Belligni E, Molinatto C, Baldassare G, Biamino E, Chiesa N, Zuffardi O, Girirajan S, Eichler EE, Ferrero GB. Eyebrow anomalies as a diagnostic sign of genomic disorders. Microdeletions and microduplications in the human genome, termed genomic disorders, contribute to a high proportion of human multisystemic neurodevelopmental diseases and are detected by array‐based comparative genomic hybridization (aCGH). In general, most genomic disorders are associated with craniofacial and skeletal features and behavioural abnormalities, in addition to learning disability and developmental delay (LD/DD). Specifically, recognition of a characteristic ‘acial gestalt’ has been the key to distinguish one genomic disorder from the other. Here, we report our experience concerning the relevance of abnormal eyebrow pattern as a diagnostic indicator of specific genomic disorders. 相似文献
92.
Tjitske Hielkema Elisa G Hamer Heleen A Reinders-Messelink Carel GB Maathuis Arend F Bos Tineke Dirks Lily van Doormaal Johannes Verheijden Carla Vlaskamp Eline Lindeman Mijna Hadders-Algra 《BMC pediatrics》2010,10(1):1-8
Background
Bronchiolitis is the most common reason for admission of infants to hospital in developed countries. Fluid replacement therapy is required in about 30% of children admitted with bronchiolitis. There are currently two techniques of fluid replacement therapy that are used with the same frequency-intravenous (IV) or nasogastric (NG). The evidence to determine the optimum route of hydration therapy for infants with bronchiolitis is inadequate. This randomised trial will be the first to provide good quality evidence of whether nasogastric rehydration (NGR) offers benefits over intravenous rehydration (IVR) using the clinically relevant continuous outcome measure of duration of hospital admission.Methods/Design
A prospective randomised multi-centre trial in Australia and New Zealand where children between 2 and 12 months of age with bronchiolitis, needing non oral fluid replacement, are randomised to receive either intravenous (IV) or nasogastric (NG) rehydration. 750 patients admitted to participating hospitals will be recruited, and will be followed daily during the admission and by telephone 1 week after discharge. Patients with chronic respiratory, cardiac, or neurological disease; choanal atresia; needing IV fluid resuscitation; needing an IV for other reasons, and those requiring CPAP or ventilation are excluded. The primary endpoint is duration of hospital admission. Secondary outcomes are complications, need for ICU admission, parental satisfaction, and an economic evaluation. Results will be analysed using t-test for continuous data, and chi squared for categorical data. Non parametric data will be log transformed.Discussion
This trial will define the role of NGR and IVR in bronchiolitisTrail registration
The trial is registered with the Australian and New Zealand Clinical Trials Registry - ACTRN12605000033640 相似文献93.
94.
95.
The identity of the enzyme(s) responsible for the S-oxidation of the mucoactive drug S-carboxymethyl-L-cysteine (SCMC) is unknown but the protein(s) are a susceptibility factor for a number of chronic degenerative diseases. The structural similarities between the amino acid L-cysteine and SCMC have raised the possibility that cysteine dioxygenase (CDO) may be responsible for this biotransformation reaction. Both CDO and SCMC S-oxygenase were found to require Fe2+ for enzymatic activity, and both enzyme activities were inhibited by Fe2+ and Fe3+ chelators. However, sulphydryl group modification of the enzymes resulted in the activation of the S-oxidation of SCMC but inhibition of the S-oxidation of L-cysteine. When the two enzyme activities were quantified in 20 female hepatic cytosolic fractions no linear correlation in the production of their respective metabolites was seen. The results of this investigation indicate that CDO is not responsible for the S-oxidation of SCMC in the rat. 相似文献
96.
Roopnarinesingh ES Steventon GB Harris RM Waring RH Mitchell SC 《Drug metabolism and drug interactions》2005,21(2):75-86
One of the major steps in the oxidation of the sulphur-containing amino acid, L-cysteine, is the production of cysteine sulphinic acid, catalysed by the enzyme cysteine dioxygenase. This enzyme plays a key role in the intermediary metabolism of sulphur-containing compounds. The activity of this crucial enzyme is known to be influenced by sulphur-compound intake, being increased in animals fed an excess of L-cysteine or methionine. However, the affects on this enzyme of the chronic administration of drugs similar in structure to cysteine are unknown. This has now been investigated using the anti-rheumatic agent, D-penicillamine, and the mucoactive compound, S-carboxymethyl-L-cysteine. Repeated oral administration of these sulphur-containing drugs to male Wistar rats for five consecutive days led to a significant increase in hepatic cysteine dioxygenase activity. This increase in the production rate of cysteine sulphinic acid remained evident until returning to control levels four days after cessation of drug administration. These observations provide evidence that these two drugs interact with the intermediary biochemistry of sulphur compounds and may provide hitherto unappreciated insights into mechanisms by which therapeutic effects and adverse reactions may occur. 相似文献
97.
Following neonatal ischaemic brain injury, irregular vessels increase in size owing to luxury perfusion. These may be demonstrated
by conventional colour flow Doppler (CFD) imaging at the periphery of the infarcted area. We present a case in which power
Doppler imaging (PDI) was performed in addition to CFD in a neonate with unexplained seizures and which proved more sensitive
than CFD in demonstrating luxury perfusion. Ultrasound appearances were compared with those seen on cranial CT. PDI can be
a useful adjunct to conventional CFD examination of the neonatal brain in cerebral infarction.
Received: 8 March 1996 Accepted: 28 April 1996 相似文献
98.
99.
Akanchha?Kesari Hanna?Rennert Debra?GB?Leonard Balraj?MittalEmail author 《BMC medical genetics》2005,6(1):22
Background
Spinal muscular atrophy (SMA) represents the second most common fatal autosomal recessive disorder after cystic fibrosis. Due to the high carrier frequency, the burden of this genetic disorder is very heavy in developing countries like India. As there is no cure or effective treatment, genetic counseling becomes very important in disease management. SMN1 dosage analysis results can be utilized for identifying carriers before offering prenatal diagnosis in the context of genetic counseling. 相似文献100.
Progesterone stimulates p42 extracellular signal-regulated kinase (p42erk) in human spermatozoa 总被引:1,自引:3,他引:1
Luconi M; Krausz C; Barni T; Vannelli GB; Forti G; Baldi E 《Molecular human reproduction》1998,4(3):251-258
Mitogen-activated protein kinases (MAPK), also known as extracellular
signal-regulated kinases (ERKs) are cytoplasmic and nuclear
serine/threonine kinases involved in signal transduction of several
extracellular effectors. Recently, we have demonstrated that ERKs are
present in spermatozoa and are involved in the regulation of the process of
capacitation. We report here the effect of progesterone, a well-known
inducer of the acrosome reaction in mammalian spermatozoa, on the
immunolocalization, phosphorylation and activity of ERKs in capacitated
human spermatozoa. We demonstrated that short-term incubation of
spermatozoa with progesterone induces phosphorylation and activation of
ERKs, resulting in redistribution of the proteins from the post-acrosomal
region to the equatorial segment within the sperm head. To investigate the
role of ERKs on the biological effects of progesterone, we used the MAPK
cascade inhibitor PD098059, which strongly inhibited progesterone-induced
activation of ERK-2. This compound did not inhibit progesterone-induced
acrosome reaction, although it prevented redistribution of the enzyme to
the equatorial region of the sperm head. These results suggest that the two
processes, although temporally related, are independent. In conclusion, we
provide new insight into the signal transduction pathways involved in the
non- genomic action of progesterone in spermatozoa and suggest a possible
involvement of ERKs in the process of fertilization.
相似文献