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排序方式: 共有113条查询结果,搜索用时 46 毫秒
41.
Lesić A Suvajdzic N Elezovic I Bumbasirevic M Hadas-Halpern I Elstein D Zimran A 《Journal of inherited metabolic disease》2006,29(4):593
Summary Extrapelvis or retroperitoneal haemorrhage has long been appreciated as having many causes and considerable variability in
subsequent morbidity; however, to date only two cases have been reported in patients with Gaucher disease, the most common
lysosomal storage disorder. It had been our assumption that these cases were unique and a consequence of severe disease in
patients who had not been treated with enzyme (or other disease-specific) therapy. Herein we present three more cases (as
well as our first patient), which allow one to make some generalizations. Ultrasound was used in one centre and computed tomography
in the second centre to make the definitive diagnosis. The trigger for the bleeding in all cases was muscle strain after activity.
All patients were young with massive hepatosplenomegaly, anaemia, thrombocytopenia, and bone pain with skeletal involvement;
the last was the most obvious commonality among these patients. Differential diagnosis is complicated by exquisite groin pain
that is common to both Gaucher disease and extrapelvis haemorrhage, but not necessarily.
Electronic supplementary material Supplementary material is available for this article at 相似文献
42.
Kroepfl T Mair E Deutsch J Brunner-Krainz M Paschke E Plecko B 《Journal of inherited metabolic disease》2006,29(4):593-593
Summary Copper-histidine is the treatment of choice in Menkes disease but bears the potential risk of copper overload and induced
liver cirrhosis. We report normal copper concentrations of liver tissue over an 8-year treatment period with copper-histidine.
Electronic supplementary material Supplementary material is available for this article at and accessible for authorised users. 相似文献
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Brautbar A Paz J Hadas-Halpern I Reinus C Rosenmann E Zimran A Elstein D 《European journal of gastroenterology & hepatology》2005,17(6):679-681
OBJECTIVES: To describe a unique case of a young man with Gaucher disease who was diagnosed with Menetrier's disease. BACKGROUND: After an acute episode of severe gastritis, the patient developed hypoalbuminemia and protein-losing gastroenteropathy, and became unwell. STUDY: Endoscopy revealed an abnormal stomach, with rigid, thickened folds covered with viscous greyish exudates. Superficial biopsies revealed foveolar hyperplasia, acute and severe gastritis with massive inflammatory infiltrate of neutrophils in the lamina propria with pit abscess formation. Tissue cultures for Helicobacter pylori were negative. RESULTS: Snare deep particle biopsy revealed the typical features of Menetrier's disease. Enzyme replacement therapy for Gaucher disease was started. CONCLUSION: This case poses a dilemma because the patient improved spontaneously, and as such is dissimilar to other adults who develop Menetrier's disease because of an infection; it is hoped that he may also not be at risk of the potential malignancies that are correlated with adult Menetrier's disease. The value of enzyme treatment is considered. 相似文献
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Wiegand I Marr AK Breidenstein EB Schurek KN Taylor P Hancock RE 《Antimicrobial agents and chemotherapy》2008,52(10):3810-3813
Screening of the PA14 genomic transposon mutant library for resistance to ceftazidime, tobramycin, and ciprofloxacin led to the discovery of several mutants that appeared in more than one screen. Testing of the frequency of mutation to ciprofloxacin resistance revealed previously known mutator genes, including mutS and mutL, as well as mutators that have not yet been described for P. aeruginosa, including PA3958 and RadA (PA4609). 相似文献
49.
Breidenstein EB Khaira BK Wiegand I Overhage J Hancock RE 《Antimicrobial agents and chemotherapy》2008,52(12):4486-4491
Pseudomonas aeruginosa offers substantial therapeutic challenges due to its high intrinsic resistance to many antibiotics and its propensity to develop mutational and/or adaptive resistance. The PA14 comprehensive mutant library was screened for mutants exhibiting either two- to eightfold increased susceptibilities (revealing genes involved in intrinsic resistance) or decreased susceptibilities (mutational resistance) to the fluoroquinolone ciprofloxacin. Thirty-five and 79 mutants with increased and decreased susceptibilities, respectively, were identified, as confirmed by broth dilution. 相似文献
50.
Mutations in the human telomerase RNA gene (TERC) cause autosomal dominant dyskeratosis congenita and have been detected in individuals with bone marrow failure. Here, we screened for TERC mutations in a cohort of 80 children with hypocellular myelodysplastic syndrome and detected TERC alterations in two of them. 相似文献