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ABSTRACT. Nation-wide neonatal screening for α1- antitrypsin deficiency (ATD) in Sweden was discontinued due to observations that identification of ATD in newborns seemed in some cases to have negative psychological effects on the parents and the parent-child relationship. A multifaceted study was developed to investigate systematically the psychological and psychosocial consequences of the identification of ATD in the neonatal period, as studied five to seven years after it took place. The study's basic goals, hypotheses, design, samples and methods are described.  相似文献   
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Autonomic nervous system activity changes have been described during cataplexy as playing a role in triggering it. To confirm these previous findings, we investigated the time course of sympathetic and cardiovascular activities during cataplexy. We made for the first time microneurographic recordings of 10 cataplectic episodes in three patients with hypocretin‐deficient narcolepsy. During microneurography, muscle sympathetic nerve activity (MSNA) was recorded simultaneously with heart rate (HR), respiratory movements, arterial finger blood pressure (BP), electroencephalography, electro‐oculogram and superficial electromyogram. Results showed no significant autonomic changes before the onset of the cataplectic episodes. Cataplexy was associated with a significant increase in MSNA and BP compared with baseline, whereas HR was markedly decreased. An irregular breathing pattern mainly characterized by apnea typically occurred during the attacks. In conclusion, our findings did not show significant changes in autonomic activity prior to cataplexy onset, ruling out a triggering role of the autonomic system. However, cataplexy was associated with co‐activation of sympathetic and parasympathetic autonomic systems, a pattern reminiscent of that reported during the vigilance reaction in animals.  相似文献   
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The cell bodies, the axons and the terminal parts of peripheral sympathetic neurons originating in the superior cervical ganglion and terminating in the dilator muscle of the iris were investigated in the rat by electron microscopy using potassium permanganate as a fixative. In all parts of the neuron granular vesicles were found. They were most numerous in the axonal enlargements of the nerve terminals, although also high numbers were found in some cell bodies, mostly localized in clusters in the peripheral parts of the cytoplasm. Two types of granular vesicles were found with a mean diameter of 495 Å (small) and 967 Å (large), respectively. The small granular vesicles were in majority, constituting about 98 per cent in the axonal enlargements in the dilator muscle of the iris. The number of vesicles in the axonal enlargements varied widely from 45 to 852 per enlargement. In all probability the small granular vesicles are the main storage site of noradrenaline in the peripheral adrenergic neurons. However, also the large ones probably contain an amine or at least are involved in the amine metabolism.  相似文献   
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Background: The entrainment mapping algorithm is used for ablation of ventricular tachycardia (VT) in right ventricular (RV) cardiomyopathy, but ablation at endocardial isthmus sites has only a moderate success rate. This study was performed to identify additional local electrogram characteristics associated with successful ablation. Patients and Methods: Using entrainment mapping, 45 reentry circuit isthmus sites were detected in 11 patients with RV cardiomyopathy presenting with 13 monomorphic VTs. Local bipolar electrograms were retrospectively analyzed at reentry circuit isthmus sites during VT, sinus rhythm, and programmed stimulation from the right ventricular apex (RVA), and compared between successful and unsuccessful ablation sites. Results: Ablation was successful at 10 reentry circuit isthmus sites and unsuccessful at 35 isthmus sites. During VT, a longer endocardial activation time relative to QRS onset, an increased electrogram‐QRS interval as a percentage of VT cycle length, and a longer electrogram duration were found at successful in comparison to unsuccessful ablation sites. The presence of isolated diastolic potentials during sinus rhythm at reentry circuit isthmus sites, consistent with slow conduction or unidirectional conduction block, was associated with successful catheter ablation. Prolongation of the duration of the local multipotential electrogram by >100 ms during programmed RVA pacing at reentry circuit exit sites, indicating functional conduction disorder was also a marker of successful ablation. Conclusions: The demonstration of multipotential electrogram characteristics indicating fixed or functional conduction block may increase the likelihood of successful VT ablation at exit and central isthmus sites of reentry circuits in RV cardiomyopathy.  相似文献   
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ABSTRACT. Immunoreactive cationic trypsin (irCT) was measured in 22 cystic fibrosis (CF) and 132 control infants. IrCT was analysed with radioimmunoassay of dried blood samples collected for PKU screening around the 5th day of life and stored on filter paper. The mean ± 1 SD level of irCT for the control infants was 42±19 μg/l. Sixteen of the 22 CF children had an irCT level above 100 μg/l (mean + 3 SD) while 6 had a level at or below this cut-off limit. A specificity of 99%, which gives a sensitivity of 73%, and an approximative noise: signal ratio of 30: 1, suggests that the irCT test may be unsatisfactory as a neonatal screening method for CF.  相似文献   
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ABSTRACT. 226 of 243 infants who took part in a prospective study of nutrition and weight during the first year of life were reviewed at age 4 years. 23 of 243 infants (9%) were obese on one or more controls the first year, and 4 of 226 (2%) at age 4 years. Only 3 of 23 infants remained obese. The weight and length of the children obsese at 0–1 year of age were significantly increased at age 4 years. Overnutrition occurred during the first year in 26 infants and the number of obese infants in this group was significantly increased at age 7–12 months and of overweight children during the first two years of life. At age 4, however, none of them were either obese or overweight.  相似文献   
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