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排序方式: 共有544条查询结果,搜索用时 31 毫秒
1.
T. Pinzer M. Reiß H. Bourquain K. G. Krishnan G. Schackert 《Acta neurochirurgica》2006,148(10):1085-1090
Summary Aspergillosis belongs to the group of mycotic diseases of paranasal sinuses. The invasive forms, and particularly the fulminant
forms, are potentially fatal. Isolated aspergillosis of the sphenoid sinus or the clivus is a difficult diagnosis, since the
often misleading clinical manifestations of this rare disease develop late. These patients become apparent by neurological
signs such as cavernous sinus syndrome, pseudotumor of the pituitary or the orbit. Diagnosis is often made intra-operatively
or on histological examination.
We report a case of invasive aspergillosis uniquely involving the sellar area revealed by clinical features suggesting a pseudotumor
of the pituitary. Although such lesions are almost always seen in immune suppressed subjects, in our case, the patient was
immune competent and had no past history of sinusitis.
The question of whether, and when to perform limited or extensive surgery remains an issue for discussion, owing to the rarity
of this disease honed by lack of experience. It depends on several factors: the kind of disease, the immunity, the subtype
of invasive fungal sinusitis and the degree of tissue invasion. 相似文献
2.
G Fitze J Cramer A Serra M Schreiber D Roesner H K Schackert 《Zeitschrift für Kinderchirurgie》2003,13(3):152-157
Hirschsprung disease (HSCR) is considered a model for a complex inheritance disorder. Several genes, including the major HSCR-susceptibility RET proto-oncogene, play an aetiological role in the development of HSCR. Genetic linkage analysis in familial HSCR with both long- and short-segment phenotypes has demonstrated a tight linkage to the RET locus, while the phenotype within a HSCR family is characterised by an incomplete penetrance or a variable extension of the aganglionosis. Therefore, additional genetic alterations of RET are postulated in the aetiology or modification of the HSCR phenotype. In this study, the coding region of all 21 exons of the RET proto-oncogene, including the flanking intronic sequences, were investigated by direct DNA sequencing in a HSCR population. We genotyped the c.135 G/A polymorphism and resolved haplotypes comprising the mutation locus and the c.135 G/A polymorphism. Twenty different mutations were detected in 18 of 76 HSCR patients. In ten families the mutations were inherited from the parents, while only four patients had a positive family history for the disease. Moreover, in all ten families an incomplete penetrance of the HSCR phenotype was observed. We have investigated the effect of the non-mutated wild-type allele as well as the c.135 G/A polymorphism on the phenotype within the HSCR families. Our findings support the notion that both RET alleles are involved in the pathogenesis of a subgroup of HSCR patients in a dose-dependent fashion. Additionally, we have shown a modifying effect of the c.135 G/A polymorphism on the HSCR phenotype within HSCR families. 相似文献
3.
4.
Arthrograms of the temporomandibular joint were obtained in 20 symptomatic joints that had previous reconstructive arthroplasty with disk repositioning because of internal derangements. Preoperative arthrograms were available for comparison in 18 joints. Symptoms resulting in a postoperative arthrogram included pain, limited ability to open the mouth, and clicking of the joints. Postoperative arthrographic findings included limited anterior translation of the condyle (90%), irregularity in outline of the intraarticular contrast agent (60%), a conical configuration of the posterior recess (25%), decreased size of the joint (28%), anterior displacement of the meniscus (25%), and perforated meniscus (15%). Many of these findings may have resulted from fibrosis and scarring, which may be a response to intraarticular bleeding. The mechanism by which the fibrosis causes the postsurgical arthrographic features is discussed. 相似文献
5.
Krüger S Bier A Plaschke J Höhl R Aust DE Kreuz FR Pistorius SR Saeger HD Rothhammer V Al-Taie O Schackert HK 《Human mutation》2004,24(4):351-352
Hereditary nonpolyposis colorectal cancer (HNPCC) is one of the most common hereditary cancer-susceptibility syndromes. Germline mutations in mismatch repair genes are associated with the clinical phenotype of HNPCC. We report ten novel germline mutations, three in MSH2 and seven in MLH1. All but one mutation have been found in families fulfilling criteria of the Bethesda guidelines; four of them additionally fulfilled the Amsterdam criteria I or II. Eight mutations were considered pathogenic and predictive diagnostics in healthy family members at risk shall be undertaken; these include five frameshift mutations leading to premature stop codons, in MSH2: c.1672delT (p.S558Xfs) and c.2466_2467delTG (p.C822X) and in MLH1: c.1023delG (p.R341Xfs), c.1127_1128dupAT (p.K377Xfs) and c.1310delC (p.P437Xfs); three mutations leading to splice aberrations, in MSH2: c.1661G>C (r.1511_1661del) and in MLH1: c.677+3A>C (r.589_677del) and c.1990-2A>G predicted to result in a splice site defect. The remaining two mutations are unclassified variants with assumed pathogenicity: one missense mutation in the highly conserved ATPase domain of MLH1 (c.122A>G [p.D41G]) and one in-frame insertion of twelve nucleotides in MLH1 (c.2155_2156insATGTGTTCCACA [p.I719delinsNVFHI]). These two mutations were not found in 102 alleles of healthy control individuals. The corresponding tumors from all patients showed a high level of microsatellite instability (MSI-H). Immunohistochemistry (IHC) revealed complete loss of expression of the affected protein in the tumor cells from all but three patients. The tumors from the patients with the mutations c.1127_1128dupAT and c.1990-2A>G showed a reduction of expression of the MLH1-protein, rather than complete loss. In the tumor from the patient with the missense mutation c.122A>G [p.D41G] a normal expression of the proteins coded by MLH1 and MSH2 was noticed. 相似文献
6.
New techniques in molecular biology and increasing insights in the genetic basis of tumorigenesis led to the development of therapeutic agents composed of genetic information. The majority of gene therapy trials is targeted towards cancer therapy. The most frequently used strategy is immunotherapy which comprises transfer of genes encoding cytokines, costimulatory molecules or tumorspecific antigens to enable cells of the immune system to identify and destroy tumor cells. The second important strategy makes use of suicide genes to selectively kill tumor cells. Compensation of genetic defects by the downregulation of activated oncogenes or the restoration of tumor suppressor gene functions is the third concept to revert the malignant phenotype of cancer cells. 相似文献
7.
Cancer development and progression has been associated with numerous genetic events in tumor cells. Germline mutations of caretaker and gatekeeper genes are responsible for hereditary cancer syndromes. Exogenous factors in conjunction with functional germline variants of a variety of genes may contribute to tumor initiation in sporadic malignant disease. Furthermore, pathways to neoplasia require somatic events in the developing tumor. Acquired or inherited genetic instability permits stepwise tumor progression. The most fearsome aspect of tumor progression is dissemination of tumor cells to draining lymph nodes of the primary or to distant organs, which limits effectiveness of surgical therapy. Cellular heterogeneity of malignant neoplasms has important implications for chemotherapy and radiotherapy. An increasing understanding of the molecular biology of tumors is the prerequisite for improved prediction, prevention and therapy of malignant disease. 相似文献
8.
9.
Han JY; Kim HK; Choi BG; Moon H; Hong YS; Lee KS 《Japanese journal of clinical oncology》1998,28(12):749-753
BACKGROUND: Quality of life (QOL) assessment has emerged to measure and
quantify the balance between treatment benefit and toxicity, and has a
value in predicting response and overall survival in cancer patients.
METHODS: From July 1995 to February 1997, 38 symptomatic patients with
advanced non-small cell lung cancer (NSCLC) were treated with MIP
chemotherapy (mitomycin 6 mg/m2, ifosfamide 3000 mg/m2 and cisplatin 50
mg/m2 on day 1 every 3 weeks). Patients were assessed for QOL including
physical well-being, general symptoms and lung cancer-specific symptoms, as
well as objective response. RESULTS: The overall response rate was 38.9%
(14/36, all were partial response) and the median duration of response was
3.5 months [95% confidence interval (CI) 2.0-4.0]. The median duration of
overall survival was 7 months (95% CI 5.9-8.5). The overall improvement of
QOL was 58.3% with 21 patients feeling better on treatment. The toxicity of
chemotherapy was mild, mainly nausea/vomiting and minimal alopecia. Using
multiple clinical predictors of survival (age, histology, stage,
performance status), only change of QOL emerged significantly (P = 0.0007).
CONCLUSIONS: MIP had an endurable response and low toxicity profile, and
provided good QOL. Integral QOL data in our study provided the strong
prediction of survival in advanced NSCLC. Further experienced QOL study
will provide greatly enhanced outcome data in clinical trials.
相似文献
10.
G. Schackert 《Der Onkologe》1998,4(7):599-607
Die malignen Gliome des Gehirns sind durch ihr infiltratives Wachstum gekennzeichnet. Der Mangel an einer Tumorkapsel und
die diffuse Ausbreitung von Tumorzellen in das umgebende Hirngewebe erschweren eine radikale Tumorexstirpation. Im Folgenden
werden die neurochirurgischen Therapiem?glichkeiten und ihre Grenzen, ein malignes Gliom durch Operation kurativ zu behandeln,
aufgezeigt. 相似文献