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排序方式: 共有441条查询结果,搜索用时 15 毫秒
1.
Expression of the neurofilament protein NF-H in L cells. 总被引:1,自引:0,他引:1
2.
The dolichol contents of urine sediments from a patient with infantile (Santavuori), a patient with late infantile (Jansky-Bielschowsky) and two patients with juvenile (Spielmeyer-Vogt) neuronal ceroid lipofuscinosis (Batten's disease) were not elevated when compared to those from healthy controls. 相似文献
3.
C. W. Chow A. Poulos A. J. Fellenberg J. Christodoulou D. M. Danks 《Acta neuropathologica》1992,83(2):190-195
Summary Recognition of adrenal atrophy during a review of autopsy findings in two sisters who died at 8 months and 3 1/2 years prompted estimation of very long chain fatty acids, phytanic acid and pristanic acid on wet liver fixed in formalin for 12 years. These were shown to be markedly increased and defects in multiple peroxisomal functions and decrease in particulate catalase were shown in cultured fibroblasts, confirming an abnormality of peroxisomal biogenesis. The patients had presented with failure to thrive, recurrent diarrohea and vomiting, poor mental development, retinal pigmentation, blindness and in the older patient deafness, with only mild dysmorphic features. Autopsy in the older patient showed adrenal atrophy, cirrhosis, and foamy histiocytes in multiple organs. The brain showed no demyelination, little cytoarchitectural abnormality, occasional perivascular histiocytes in the grey matter and meninges and prominent Purkinje cells in the molecular layer of the cerebellum. In the younger patient the changes were very subtle in spite of the marked clinical similarity. Despite the young age at death the clinicopathological features are most suggestive of infantile Refsum disease. In many situations anatomical pathology can be very useful in the recognition and study of peroxisomal disorders. 相似文献
4.
H. Coughtrey H. E. Jeffery D. J. Henderson-Smart B. Storey V. Poulos 《The Australian & New Zealand journal of obstetrics & gynaecology》1991,31(2):97-102
The cause of fetal distress and neonatal respiratory distress (RD) in association with meconium-stained liquor is not always clear. To clarify this, a prospective study was undertaken in a tertiary referral maternity hospital for 1 year. In all infants born after meconium-stained liquor who developed RD, evidence was sought for 1) fetal distress (from the cardiotocograph (CTG), the cord blood pH, the Apgar score and the asphyxial complications in the neonate) 2) causes of fetal distress (including maternal risk factors, fetal infection and fetal malnutrition) 3) causes of respiratory distress (including meconium aspiration syndrome (MAS), persistent pulmonary hypertension of the newborn (PPHN) and infection). Of 4,026 livebirths, 717 (17.8%) had meconium-stained liquor and 44 term and 5 preterm infants developed RD. In the 44 term infants, there was frequent evidence of fetal distress possibly caused by previously unrecognized factors such as fetal malnutrition with reduced neonatal skinfold thickness in 35% triceps and 41% subscapular measurements, and histological chorioamnionitis (CA) in 74%. The cause for respiratory distress was identified in only 48% of infants, and included clinical evidence of PPHN (41%), MAS (16%) and infection (2%). However in preterm infants, 80% had definite or suspected infection. The findings indicate that fetal distress is common in infants who develop respiratory distress after meconium-stained liquor. A role for histological CA and reduced nutrition in the fetus, as factors contributing to the vulnerability of the term infant to intrapartum fetal distress, is suggested. 相似文献
5.
Poulos LM O'Meara TJ Hamilton RG Tovey ER 《The Journal of allergy and clinical immunology》2002,109(4):701-706
BACKGROUND: IgE-mediated responses to natural rubber latex allergens have become a major health problem among recognized risk groups. OBJECTIVE: The purpose of this investigation was to measure the amounts of Hevea brasiliensis latex allergen (Hev b 1) inhaled and deposited on surfaces when latex or vinyl gloves were worn and compare the results with the conventional measures (breathing zone samplers) of occupational exposure. METHODS: Hev b 1 exposure was measured by nasal sampling and breathing zone sampling. Latex allergen exposure was generated by having each subject don a pair of powdered latex examination gloves and continuing his or her normal daily activity for 30 minutes. By means of adhesive tape, surface dust samples were collected from the surfaces of gloves, the subject's hands, and work areas. Sampling was performed with subjects wearing no gloves, subjects wearing powdered vinyl gloves, subjects wearing powdered latex gloves, and nearby colleagues wearing latex gloves. All samples were assayed through use of the HALOgen assay (Inhalix, Sydney, Australia) with a Hev b 1-specific mAb. Particles transporting latex allergen were identified by a surrounding immunostain halo, and these were quantified and reported as total numbers of particles inhaled, airborne, or found on surface areas evaluated. RESULTS: Study subjects inhaled 26 times more allergen when powdered latex gloves were worn than under the "no glove" and powdered vinyl glove conditions. During the same period, Hev b 1 particle levels measured in the ambient air through use of the breathing zone sampler increased by 24-fold. The median numbers of particles carrying Hev b 1 allergen per square centimeter on the surface of the hands after the wearing of latex and vinyl gloves were 1964 and 5, respectively. Latex allergen was physically associated both with cornstarch granules and with larger dust particles having a darker, more irregular appearance. CONCLUSION: In a laboratory where gloves are worn for protection, the use of latex gloves resulted in a 26-fold increase in inhaled latex allergen over background levels measured while vinyl gloves were worn as controls. Low levels of latex exposure also occurred when vinyl gloves or no gloves were worn; the reasons for this are under investigation. 相似文献
6.
Localization of plasminogen activator and inhibitor, LH and androgen receptors and inhibin subunits in monkey epididymis 总被引:4,自引:1,他引:4
Epididymis is a site of sperm maturation and storage. Limited and
directed-proteolysis regulated by plasminogen activator (PA), plasminogen
activator inhibitor type-1 (PAI-1) and other related factors may play an
essential role in these processes. Our previous studies have demonstrated
that rat epididymis expressed luteinizing hormone receptor (LHR), tissue
type (t) and urokinase type (u)PA, mRNAs, and tPA activity was stimulated
in vitro by human chorionic gonoadotrophin (HCG). In the present study we
further examined localization of mRNAs for tPA, uPA, LHR, androgen receptor
(AR), as well as inhibin subunits alpha, betaA and betaB in rhesus monkey
epididymis. Using in-situ hybridization with digoxygenin-labelled cRNA
probes, we have demonstrated that tPA and PAI-1 mRNAs were localized in
epithelial cells of adult monkey epididymis. uPA mRNA was localized in the
same areas, but to a much smaller extent. tPA, uPA and PAI-1 mRNAs were
greatly expressed in the caput and corpus of adult epididymis than in other
regions. In-vitro experiments showed that both tPA and uPA activities in
epididymal cells were dramatically stimulated by HCG, but not by follicle
stimulating hormone (FSH). LHR (but not FSH receptor) and AR mRNAs were
localized in the epithelial cells of the epididymis. However, LHR mRNA was
detected in both adult and immature infant monkeys, whereas AR was found
only in the adult. Inhibin alpha, betaA and betaB mRNAs were also detected
in this organ, betaA mRNA being more strongly expressed in the caput than
in other regions of the epididymis. We suggest that LH and androgen may be
the key hormones in coordination with the PA-PAI-1 system in regulating
epididymal differentiation and sperm maturation.
相似文献
7.
The accumulation of very long chain fatty acids in plasma and skin fibroblasts was measured in at least four separate inherited disease states. Both the magnitude and the nature of the fatty acid changes reflected the clinical status of individual patients. In Zellweger's syndrome, and to a lesser extent in infantile Refsum's disease, there was an increase in 24:0, 26:0, 26:1, and a number of even longer chain fatty acids, while in the X-linked form of adrenoleukodystrophy these changes were less pronounced. Zellweger fibroblasts in culture took up lignoceric, phytanic and stearic acids and incorporated them into a variety of lipids in a manner comparable to control fibroblasts. However, these cells were unable to convert phytanic or lignoceric acid to CO2. Infantile Refsum's and X-linked adrenoleukodystrophy fibroblasts showed normal conversion of these acids to CO2. Normal fibroblast homogenates produced radioactive acetate from [1-14C] stearic and [1-14C] lignoceric acids indicating that both substrates were beta-oxidised under these conditions. Homogenates of fibroblasts from all patients patients with biochemical evidence of accumulation of very long chain fatty acids showed normal or near-normal stearic acid beta-oxidation, but were deficient in lignoceric acid beta-oxidation. Residual lignoceric acid beta-oxidation activity varied from approximately 15% in Zellweger syndrome up to 50% in X-linked adrenoleukodystrophy. It is postulated that the accumulation of very long chain fatty acids results from defects in peroxisomal beta-oxidation. In Zellweger's syndrome, and possibly in infantile Refsum's disease, it is probable that this defect is secondary to a primary abnormality affecting the structure and/or function of peroxisomes, while the primary defect in X-linked adrenoleukodystrophy may be confined to a pathway specific for the oxidation of very long chain fatty acids. 相似文献
8.
Identification of the multiple endocrine neoplasia type 1 (MEN1) gene. The European Consortium on MEN1 总被引:10,自引:0,他引:10
Lemmens I; Van de Ven WJ; Kas K; Zhang CX; Giraud S; Wautot V; Buisson N; De Witte K; Salandre J; Lenoir G; Pugeat M; Calender A; Parente F; Quincey D; Gaudray P; De Wit MJ; Lips CJ; Hoppener JW; Khodaei S; Grant AL; Weber G; Kytola S; Teh BT; Farnebo F; Thakker RV 《Human molecular genetics》1997,6(7):1177-1183
9.
Each of 28 Vibrio strains and 3 Campylobacter strains was immobilized in distilled water (pH 6.9), but retained full motility in Trypticase soy broth. This phenomenon was not seen with any of 243 non-Vibrio, non-Campylobacter isolates representing 44 taxa of gram-negative bacteria. 相似文献
10.