Children with urea cycle disorders present with hyperammonaemiaand its non-specific symptoms. Acute hyperammonaemia is a medicalemergency as the developmental and neurological outcome dependson the duration of hyperammonaemic coma [1]. The longer endogenousprotein catabolism continues, the more ammonia will be producedand accumulate and the greater is the risk of coma. To minimizepermanent brain damage, early diagnosis and appropriate therapyis mandatory. With diagnosis of hyperammonaemia, it is essentialto differentiate between urea cycle defects and other causesof encephalopathy. In Figure 1 a practicable flowchart for establishingthe correct diagnosis is depicted [2]. The emergency therapyin children with inborn metabolic disorders presenting  相似文献   
167.
168.
CT-guided high-dose-rate brachytherapy of unresectable hepatocellular carcinoma     
Collettini  Federico  Schreiber  Nadja  Schnapauff  Dirk  Denecke  Timm  Wust  Peter  Schott  Eckart  Hamm  Bernd  Gebauer  Bernhard 《Strahlentherapie und Onkologie》2015,191(5):405-412
Strahlentherapie und Onkologie - The purpose of the present study was to evaluate the clinical outcome of CT-guided high-dose-rate brachytherapy (CT-HDRBT) in patients with unresectable...  相似文献   
169.
Gadoxetic acid enhanced MRI for differentiation of FNH and HCA: a single centre experience     
Christian Grieser  Ingo G. Steffen  Incken-Birthe Kramme  Hendrik Bläker  Ergin Kilic  Carmen Maria Perez Fernandez  Daniel Seehofer  Eckart Schott  Bernd Hamm  Timm Denecke 《European radiology》2014,24(6):1339-1348

Objectives

Evaluation of enhancement characteristics of histopathologically confirmed focal nodular hyperplasias (FNHs) and hepatocellular adenomas (HCAs) with gadoxetic acid-enhanced MRI.

Methods

Sixty-eight patients with 115 histopathologically proven lesions (FNHs, n?=?44; HCAs, n?=?71) examined with gadoxetic acid-enhanced MRI were retrospectively enrolled (standard of reference: surgical resection, n?=?53 patients (lesions: FNHs, n?=?37; HCAs, n?=?53); biopsy, n?=?15 (lesions: FNHs, n?=?7; HCAs, n?=?18)). Two radiologists evaluated all MR images regarding morphological features as well as the vascular and hepatocyte-specific enhancement in consensus.

Results

For the hepatobiliary phase, relative enhancement of the lesions and lesion to liver enhancement were significantly lower for HCAs (mean, 48.7 (±48.4) % and 49.4 (±33.9) %) compared to FNHs (159.3 (±92.5) %; and 151.7 (±79) %; accuracy of 89 % and 90 %, respectively; P?<?0.001). Visual strong uptake of FNHs vs. hypointensity of HCAs in the hepatobiliary phase resulted in an accuracy of 92 %. This parameter was superior to all other morphological and dynamic vascular criteria alone and in combination (accuracy, 54–85 %).

Conclusions

For differentiation of FNHs and HCAs by means of MRI, gadoxetic acid uptake in the hepatobiliary phase was found to be superior to all other criteria alone and in combination.

Key Points

? EOB-MRI is well suited to differentiate FNHs and hepatocellular adenomas. ? For this purpose hepatobiliary phase is superior to unenhanced and dynamic imaging. ? Hepatobiliary phase (peripheral) hyper- or isointensity is typical for FNH. ? Hepatobiliary phase hypointensity is typical for hepatocellular adenomas. ? EOB-MRI helps to avoid misinterpretations of benign hepatocellular lesions.  相似文献   
170.
Composite aortic root replacement in acute type A dissection: time to rethink the indications?     
James C Halstead  David Spielvogel  Dieter M Meier  Sindy Rinke  Carol Bodian  Ramin Malekan  M Arisan Ergin  Randall B Griepp 《European journal of cardio-thoracic surgery》2005,27(4):626-32; discussion 632-3
OBJECTIVE: The indications for aortic root replacement in acute type A dissection are unclear. We reviewed the immediate and long-term outcome of consecutive patients in a series in which a low-threshold policy of composite aortic root replacement had evolved. METHODS: From a prospectively compiled aortic surgery database, we identified 162 patients who had either supracoronary interposition grafting, Group A (n=89), or composite root replacement, Group B (n=73) for acute type A dissection. Patients receiving total arch replacements were excluded. Operative and clinical details were analyzed and patient survival was compared to an age and gender matched census cohort. Need for reoperation on the proximal or distal aorta was also noted. Follow-up totaled 795.5 patient-years. RESULTS: Hospital mortality rates were identical in both groups (12.3%: 11 deaths in group A; 9 in group B). Chronic pulmonary disease, diabetes, malperfusion, hemodynamic compromise and aortic root dilatation were independent risk factors for hospital death. Actuarial survival estimates at 1, 5 and 10 years were 79% (71-88%), 64% (53-75%), and 55% (41-68%) for group A, and 79% (70-86%), 73% (62-83%), and 65% (52-78%) for group B (P=0.48). Age and operative patency of the ascending false lumen were independent risk factors for death after hospital discharge. Proximal aortic reoperation was required for four patients in group A and none in group B (P=0.085). CONCLUSION: A strategy of replacement rather than repair of the dissected aortic root for specific indications in type A dissection yielded high survival and low proximal reoperation rates. These results support an aggressive policy of composite root replacement in acute type A dissection.  相似文献   
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161.
The mushroom body of the insect brain is an important locus for olfactory information processing and associative learning. The present study investigated the biophysical properties of Kenyon cells, which form the mushroom body. Current- and voltage-clamp analyses were performed on cultured Kenyon cells from honeybees. Current-clamp analyses indicated that Kenyon cells did not spike spontaneously in vitro. However, spikes could be elicited by current injection in approximately 85% of the cells. Of the cells that produced spikes during a 1-s depolarizing current pulse, approximately 60% exhibited repetitive spiking, whereas the remaining approximately 40% fired a single spike. Cells that spiked repetitively showed little frequency adaptation. However, spikes consistently became broader and smaller during repetitive activity. Voltage-clamp analyses characterized a fast transient Na+ current (INa), a delayed rectifier K+ current (IK,V), and a fast transient K+ current (IK,A). Using the neurosimulator SNNAP, a Hodgkin-Huxley-type model was developed and used to investigate the roles of the different currents during spiking. The model led to the prediction of a slow transient outward current (IK,ST) that was subsequently identified by reevaluating the voltage-clamp data. Simulations indicated that the primary currents that underlie spiking are INa and IK,V, whereas IK,A and IK,ST primarily determined the responsiveness of the model to stimuli such as constant or oscillatory injections of current.  相似文献   
162.
There are two popular strategies for current drug treatment of epilepsy; starting early may be better and polytherapy conveys advantages over monotherapy. This review briefly examines if the historical record is much of a guide to determine the clinical value of these two strategies. Great clinical scientists of the 19th and early 20th century, such as Sir William Gowers, and William Aldren Turner, offered vivid single case studies and showed early results of seizure remission in groups of subjects. The historical record offered, however, no evidence of clear clinical benefits for early treatment and polytherapy. Combination treatment was thought to be useful in only some cases. In agreement, current evidence shows no clear clinical benefit of starting treatment early, except perhaps in severe epilepsy. Polytherapy is clinically useful in a subgroup of subjects, but despite being a standard treatment strategy for over one hundred years, it has been poorly studied. In fact, there is no compelling experimental or clinical evidence for a difference in seizure outcome between monotherapy and polytherapy. This surprising finding should prompt a re‐appraisal regarding the need to test both strategies separately for the licensing of new antiepileptic drugs.  相似文献   
163.

Purpose  

In the last 15 years, vertebroplasty and kyphoplasty have become established operative procedures for treating osteoporotic vertebral-body fractures and vertebral bodies afflicted with metastases. These procedures are quickly performed with few personnel and material resources and have a low rate of complications. However, cases of neurological impairment are reported in the scientific literature. We analysed whether potentially harmful heat is radiated/conducted by the polymerisation temperature of polymethylmethacrylate (PMMA) bone cement in the spinal canal.  相似文献   
164.
To report a combined ascending aorta and aortic arch hybrid repair, we performed off-pump with no aortic graft replacement. A 65-year-old man, developing progressive recurrent laryngeal nerve paralysis, underwent a computed tomography (CT) angiography detecting nonpatent residual ductus Botalli aneurysm and ascending aorta aneurysm. Due to severe multimorbidities, a less-invasive alternative was elaborated. In a first step, appropriate proximal landing zone for aortic stent grafting was achieved by ascending aorta diameter reduction, with epiaortic wrapping, and debranching the supra-aortic trunks. In the second step, endovascular stent grafts were deployed from proximal ascending aorta to descending aorta, excluding the ductus Botalli aneurysm. Six-month follow-up CT shows ductus Botalli aneurysm exclusion, stable stent graft position, and ascending diameter and patent and stenosis-free supra-aortic grafts. This case supports this alternative treatment to open aortic repair under circulatory arrest and deep hypothermia, especially in those patients considered unfit for such invasive treatment.  相似文献   
165.
Plasmacytoid urothelial carcinoma of the bladder represents a rare and aggressive variant of urothelial carcinoma, which is usually diagnosed at an advanced pathologic stage. Until now, no reports exist on this rare tumor type in the upper urinary tract. Herein, we present the first report on the clinical course of a metastatic plasmacytoid urothelial carcinoma of the renal pelvis and show its unfavorable outcome despite multimodal therapy.  相似文献   
166.
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