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排序方式: 共有280条查询结果,搜索用时 15 毫秒
1.
Franck Ceppa Stephane Gidenne Alain Benois Eleonore Fontan Pascal Burnat 《Clinical chemistry and laboratory medicine》2002,40(8):799-801
Human butyrylcholinesterase is the enzyme responsible of mivacurium and succinylcholine metabolism, which may be significantly impaired when mutation Asp70Gly is found in patients. We describe a simple PCR method for the detection of this variant. Thirteen out of sixteen patients tested after prolonged apnea were positive for the presence of this mutation (50.0% homozygotes and 31.3% heterozygotes), suggesting that this test contributes to the explanation of some clinical events and to their prevention in relatives of these patients. 相似文献
2.
The prognostic significance of acute renal failure after renal transplantation in patients treated with cyclosporin 总被引:2,自引:0,他引:2
Perez Fontan M; Rodriguez-Carmona A; Bouza P; Valdes F 《QJM : monthly journal of the Association of Physicians》1998,91(1):27-40
We studied 733 cadaveric renal transplant patients (747 transplants) under
cyclosporin immunosuppression, to: (i) establish the risk profile for acute
renal failure (ARF) after renal transplantation in a unit using many
sub-optimal donors; (ii) assess the long-term prognostic relevance of ARF;
and (iii) explore the synergistic prognostic significance of delayed graft
function and acute rejection during the early post-transplant period.
Transplanting from a non-heart-beating or elderly donor, protracted cold
ischaemia, haemodialysis immediately before transplant surgery, poor HLA
matching, and grafting to a hypersensitized recipient without residual
renal function, all independently predicted delayed graft function. This
delay had no detrimental effect on patient or graft survival, but prolonged
ARF was associated with increased mortality from infection. Late markers of
graft dysfunction (poor graft function, proteinuria, hypertension) were
highly prevalent among grafts affected by ARF, specially in prolonged ARF.
Delayed graft function and early acute rejection showed a definite, albeit
not strong, additive impact on late graft survival, and also on the
prevalence of late markers of graft dysfunction.
相似文献
3.
Surgical Treatment of an Early Epileptic Encephalopathy with Suppression-Bursts and Focal Cortical Dysplasia 总被引:3,自引:3,他引:0
Summary: A case of early epileptic encephalopathy (EIEE) with suppression-bursts or Ohtahara's syndrome, associated with focal cortical dysplasia is reported. Infantile spasms and brief tonic unilateral seizures began on the fifth day of life. Interictal EEG demonstrated an asymmetrical "suppression-burst" pattern with no wake or sleep cycling. Seizures were refractory to all antiepileptic drug (AED) and steroid therapy. Magnetic resonance imaging (MRI) showed right frontotemporal cortical thickening. After three weeks of an ineffective medical treatment a preoperative evaluation with single photon emission computed tomography (SPECT) and electrocorticography (ECoG) was performed to characterize epileptogenic focus. Surgical resection of the precentral area was performed at age 1 month. Neuropathologic examinations confirmed diagnosis of focal cortical dysplasia by identifying cytoarchitectural disarray and ectopic neurons located deep in subcortical white matter. During follow-up, 1-year postoperative the child had a single febrile seizure. Neurologic examination showed minor developmental delay and slight left-sided weak ness. 相似文献
4.
N. Matamoros Florí J. Mila Llambi T. Español Boren S. Raga Borja G. Fontan Casariego 《Journal of clinical immunology》1997,17(4):333-339
The Spanish Registry for Primary Immunodeficiency Diseases (REDIP) was organized in 1993. One thousand sixty-nine cases of primary immunodeficiency diseases (PID) were registered in patients diagnosed between January 1980 and December 1995. PID diagnosis was made according to the World Health Organization criteria. The most frequent disorders were IgA deficiency (n = 394) and common variable immunodeficiency (n = 213), followed by severe combined immunodeficiency (n = 61), C1 inhibitor deficiency (n = 52), X-Iinked agammaglobulinemia (n = 49), IgG subclass deficiency (n = 48), and chronic granulomatous disease (n = 32). A comparative study between REDIP and data recently obtained from the European registry (ESID Report, 1995) revealed important differences between phagocytic disorders and complement deficiencies reported in both registries, 4.9 vs 8.7 and 6.0 vs 3.6, while percentages of predominantly antibody deficiencies and T cell and combined deficiencies concurred with those reported in the European registry, 69.3 vs 64.7 and 14.7 vs 20.2, respectively. The heterogeneous nature of the geographical distribution of cases submitted may indicate underdiagnosis of PID in some country areas; surprisingly, the interval between the onset of clinical symptoms and diagnosis was significant, even in immunodeficiency diseases, such as IgA deficiency, which are easy to diagnose. 相似文献
5.
O G Segurado A A Arnaiz-Villena P Iglesias-Casarrubios J Martinez-Laso J L Vicario G Fontan M Lopez-Trascasa 《Clinical and experimental immunology》1992,87(3):410-414
The first inherited combined total deficiency of C7 and C4B complement components associated with SLE is described in a young female. Functional C7 assays showed a homozygous C7 deficiency in the propositus and her sister, and an heterozygous one in their parents. C4 molecular analyses showed that both the propositus and her mother had two HLA haplotypes carrying only C4A-specific DNA sequences and a normal C4 gene number. Thus, only C4A proteins could be expressed, with resultant normal C4 serum levels. The coexistence of a combined complete C7 and C4B deficiency may therefore abrogate essential functions of the complement cascade presumably related to immune complex handling and solubilization despite an excess of circulating C4A. These findings challenge the putative pathophysiological roles of C4A and C4B and stress the need to perform both functional assays and C4 allotyping in patients with autoimmune pathology and low haemolytic activity without low serum levels of a classical pathway complement component. 相似文献
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Performing a preliminary hazard analysis applied to administration of injectable drugs to infants 下载免费PDF全文
10.
One case of subarachnoid haemorrhage due to a spinal cord tumor, seen in a child, is reported. This exceptional etiology, whose the look is often misleading must be called to mind when meningeal and rachidian syndromes are associated. Ependymoma of cauda equina is the most common tumor. 相似文献