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91.
NC Voermans J Timmermans N van Alfen S Pillen J op den Akker M Lammens MJ Zwarts IALM van Rooij BC Hamel BG van Engelen 《Clinical genetics》2009,76(1):25-37
Marfan syndrome is a clinically and allelic heterogeneous, heritable connective tissue disorder with infrequently reported neuromuscular features. This study is the first to delineate these symptoms in a non-selected population. Neuromuscular involvement was evaluated in 10 Marfan patients through a standardized questionnaire, physical examination, nerve conduction study (NCS), needle electromyography (EMG), muscle ultrasound, laboratory investigation, and muscle biopsy. Existing neuroimages were screened for dural ectasia and spinal meningeal cysts. Twenty healthy controls with similar age distribution completed the questionnaire.
The results showed that various neuromuscular symptoms occur more frequently in the patients. Four older patients reported muscle weakness, five patients had a mild-to-moderate reduction in vibration sense, and all older patients mentioned mild functional impairments. NCS showed axonal polyneuropathy in four and EMG myopathic and neurogenic changes in all patients. Increased echo intensity and atrophy on muscle ultrasound was found in more than half of the patients. Muscle biopsies obtained in two patients showed myopathic changes in the older, female patient.
In conclusion, the majority of Marfan patients exhibited neuromuscular symptoms characterized as myopathy or polyneuropathy or both, and signs of lumbosacral radiculopathy, with symptoms being most pronounced in the older patients. Although meriting corroboration, these findings indicate a need to further the awareness of neuromuscular involvement in this population. 相似文献
The results showed that various neuromuscular symptoms occur more frequently in the patients. Four older patients reported muscle weakness, five patients had a mild-to-moderate reduction in vibration sense, and all older patients mentioned mild functional impairments. NCS showed axonal polyneuropathy in four and EMG myopathic and neurogenic changes in all patients. Increased echo intensity and atrophy on muscle ultrasound was found in more than half of the patients. Muscle biopsies obtained in two patients showed myopathic changes in the older, female patient.
In conclusion, the majority of Marfan patients exhibited neuromuscular symptoms characterized as myopathy or polyneuropathy or both, and signs of lumbosacral radiculopathy, with symptoms being most pronounced in the older patients. Although meriting corroboration, these findings indicate a need to further the awareness of neuromuscular involvement in this population. 相似文献
92.
C Dye K L?nnroth E Jaramillo BG Williams M Raviglione 《Bulletin of the World Health Organization》2009,87(9):683-691
Objective
To determine whether differences in national trends in tuberculosis incidence are attributable to the variable success of control programmes or to biological, social and economic factors.Methods
We used trends in case notifications as a measure of trends in incidence in 134 countries, from 1997 to 2006, and used regression analysis to explore the associations between these trends and 32 measures covering various aspects of development (1), the economy (6), the population (3), behavioural and biological risk factors (9), health services (6) and tuberculosis (TB) control (7).Findings
The TB incidence rate changed annually within a range of ±10% over the study period in the 134 countries examined, and its average value declined in 93 countries. The rate was declining more quickly in countries that had a higher human development index, lower child mortality and access to improved sanitation. General development measures were also dominant explanatory variables within regions, though correlation with TB incidence trends varied geographically. The TB incidence rate was falling more quickly in countries with greater health expenditure (situated in central and eastern Europe and the eastern Mediterranean), high-income countries with lower immigration, and countries with lower child mortality and HIV infection rates (located in Latin America and the Caribbean). The intensity of TB control varied widely, and a possible causal link with TB incidence was found only in Latin America and the Caribbean, where the rate of detection of smear-positive cases showed a negative correlation with national incidence trends.Conclusion
Although TB control programmes have averted millions of deaths, their effects on transmission and incidence rates are not yet widely detectable. 相似文献93.
A new technique of intracavitary brachytherapy for malignant biliary obstruction is presented. The technique involves the use of a high-dose-rate remote afterloading device, which offers all the advantages of conventional brachytherapy with the added benefit that the dose can be delivered in a single treatment over a few minutes. The potential problems associated with conventional brachytherapy are thereby minimized. 相似文献
94.
95.
Human myeloma in vitro colony growth: interrelationships between drug sensitivity, cell kinetics, and patient survival duration 总被引:1,自引:0,他引:1
Ninety-seven patients with multiple myeloma evaluated serially had both a tritiated thymidine labeling index of bone marrow plasma cells (LI%) and in vitro myeloma stem cell culture performed. Thirty-three patients with myeloma colony growth had in vitro drug sensitivity testing carried out, 18 having in addition in vitro thymidine suicide determinations. The LI% and the likelihood of in vitro myeloma colony growth were highly correlated: the higher the LI%, the more likely was colony or cluster growth (p less than 0.001). The tritiated thymidine suicide of myeloma stem cells was usually very high. There was excellent correlation between in vitro and in vivo drug sensitivity. Both pretreatment drug resistance and selective sensitivity (e.g., interferon, bisantrene, methotrexate, vinblastine) at the time of relapse were accurately detected and correlated well with survival duration (p = 0.01 Wilcoxan). Although LI% and in vitro sensitivity were clearly independent variables, a high LI% (greater than 3%) plus in vitro resistance were associated with a subsequent survival duration of less than 6 mo. The studies allowed dissection of the complex interrelationship between cell kinetics and drug sensitivity. 相似文献
96.
Anthony?F?JormEmail author Betty?A?Kitchener Richard?O'Kearney Keith?BG?Dear 《BMC psychiatry》2004,4(1):33
Background
A Mental Health First Aid course has been developed which trains members of the public in how to give initial help in mental health crisis situations and to support people developing mental health problems. This course has previously been evaluated in a randomized controlled trial in a workplace setting and found to produce a number of positive effects. However, this was an efficacy trial under relatively ideal conditions. Here we report the results of an effectiveness trial in which the course is given under more typical conditions. 相似文献97.
JH Cross GD Jackson BG Neville A Connelly FJ Kirkham SG Boyd MC Pitt DG Gadian 《Archives of disease in childhood》1993,69(1):104-109
The incidence of brain abnormalities determined by magnetic resonance in 30 consecutive children presenting with intractable complex partial seizures is reported. Images were optimised to visualise the hippocampus and cortical grey matter. Abnormalities of the hippocampus or temporal lobe were seen in all 19 children with clinical features of temporal lobe epilepsy and in six of the seven children with clinically unlocalised epilepsy. By contrast, in the four children with a clinical diagnosis of extratemporal epilepsy, no temporal or hippocampal abnormalities were seen. Generalised cortical abnormalities of uncertain significance were found in a total of 14 children from all groups. The identification of focal brain abnormalities using optimised magnetic resonance imaging enables early non-invasive assessment of children with intractable seizure disorders and the identification of patients for whom epilepsy surgery may be appropriate. It may also lead to a better understanding of the structural basis of intractable epilepsy, and thereby contribute to early treatment decisions. 相似文献
98.
AA Alarabi BG Danielson B Wikström A Kreuger G Tufveson 《Acta paediatrica (Oslo, Norway : 1992)》1992,81(1):75-78
This case report describes a special management approach in a child aged 4.5 years with a severe form of hepatorenal syndrome in which the final diagnosis was familial hemophagocytic lymphohistiocytosis (FHL). The patient presented with grade IV hepatic coma and acute renal failure (ARF). While the diagnosis was difficult at the time of admission, as well as during acute treatment, artificial liver support was established for elimination of bilirubin and other metabolic by-products by using charcoal column plasma perfusion (CCPP) and bilirubin-adsorbing resin column plasma perfusion (BRCPP). Serum levels of bilirubin showed a notable decrease after each of the four treatment sessions. Additional artificial renal function replacement by continuous arteriovenous hemofiltration and hemodialysis (CAVH, CAVHD, respectively) had a marked lowering effect on urea and creatinine serum concentrations. Both artificial liver and renal support contributed to the general clinical improvement and survival of the patient. Further experience in these therapies will be needed to establish better prognosis in such fatal or acute similar conditions. 相似文献
99.
Twin and singleton growth patterns compared using US 总被引:1,自引:0,他引:1
Sonography has been used widely in the evaluation of singleton fetal growth. Twin gestations, however, have received less careful attention. In a statistical study of 103 twin pregnancies, the growth patterns of twin biparietal diameter (BPD), fetal femur length (FFL), and abdominal circumference (AC) were compared with those of singletons. The results of the study revealed a decrease in twin BPD growth after 31 to 32 weeks of gestation relative to singletons. Twin AC growth rate decreases after 32-33 weeks of gestation relative to singletons, but the twin FFL growth pattern does not deviate from that of singletons throughout gestation. Because of the significant difference in growth patterns of BPD and AC between twins and singletons in our population, new growth charts for twin BPD and AC are proposed. 相似文献
100.