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Zehra Narli Ozdemir Guldane Cengiz Seval Ugur Sahin Atilla Uslu Mehmet Gunduz Sinem Civriz Bozdag Selami Kocak Toprak Meltem Kurt Yuksel Pervin Topcuoglu Isinsu Kuzu Muhit Ozcan Gunhan Gurman Osman Ilhan 《Indian journal of hematology & blood transfusion》2021,37(1):67
PurposeBlastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and poor prognostic hematological malignancy. There is still no standard treatment established for BPDCN patients. We aim to summarize the main clinical, biological features and treatment of 9 BPDCN patients.MethodsNine patients with BPDCN who had been diagnosed between July 2008 and December 2018 in Ankara University School of Medicine, were retrospectively evaluated.ResultsAll patients (n = 9) were male, median age was 64 (21–80). Five patients (55.6%) had bone marrow infiltration, 5 patients (55.6%) cutaneous lesions, 6 patients (66.7%) lymph node involvement, 2 patients (22.2%) central nervous system involvement and 2 patients (22.2%) spleen involvement at time of diagnosis. Complex karyotype was observed in 2 patients. CHOP was given to 5 patients (55.6%), hyper-CVAD to 2 patients (22.2%), fludarabine, cyclophosphamide and mitoxantrone to 1 patient (11.1%) and cyclophosphamide, etoposide, methylprednisolone to 1 patient (11.1%) as first line chemotherapy. Four patients (44.4%) underwent allogeneic hematopoietic stem cell transplantation (AHSCT) in complete remission (CR) 1. Venetoclax was given to a transplant ineligible patient who had skin and lymph node involvement, with the off-label use. The median follow-up time was 15.9 months (3–48.6 months). Estimated median overall survival was 15.9 + 1.6 (95% CI 12.7–19.1) months.ConclusionIntensive induction therapies followed by AHSCT in CR seems to be best approaches for patients with BPDCN. Thus, more effective treatment strategies particularly targeted therapies should be warranted to improve the survival of patients with this rare disease. 相似文献
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The aim of this study was to evaluate the total antioxidant status (TAS), total oxidative status (TOS) and oxidative stress
index (OSI) in patients with postmenopausal osteoporosis. We also investigate the relation between bone mineral density and
oxidative/antioxidative parameters. Thirty-nine patients with osteoporosis and 26 healthy controls were included in the study.
Plasma TAS, TOS levels were determined by using a novel automated methods. Plasma TOS and OSI value were significantly higher,
and plasma TAS level was lower in patients than in healthy controls (P < 0.001 for all). There was a significant negative correlation between OSI and BMD in lumbar and femoral neck region (r = −0.63, P < 0.001; r = 0.40, P = 0.018). The results of this study indicated that increased osteoclastic activity and decreased osteoblastic activity may
be associated with an imbalance between oxidant and antioxidant status in postmenopausal osteoporosis. Therefore, supplementation
of antioxidant-enriched diet to the therapy might shed light on the development of novel therapeutic strategies for osteoporosis. 相似文献
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Hande Ikitimur Betul Borku Uysal Mahir Cengiz Bar Ikitimur Harun Uysal Erkan Ozcan Mehmet Sami Islamoglu Serhat Seyhan Hakan Yavuzer Serap Yavuzer 《Journal of medical virology》2021,93(1):357-365
In this study, we report a large family cluster consisting of 29 genetically related patients hospitalized with coronavirus disease‐2019 (COVID‐19). We sought to determine the clinical characteristics relevant to the clinical course of COVID‐19 by comparing the family cluster to unrelated patients with SARS‐CoV‐2 infection so that the presence of potential determinants of disease severity, other than traditional risk factors previously reported, could be investigated. Twenty‐nine patient files were investigated in group 1 and group 2 was created with 52 consecutive patients with COVID‐19 having age and gender compatibility. The virus was detected for diagnosis. The clinical, laboratory and imaging features of all patients were retrospectively screened. Disease course was assessed using records regarding outcome from patient files retrospectively. Groups were compared with respect to baseline characteristics, disease severity on presentation, and disease course. There was no difference between the two groups in terms of comorbidity and smoking history. In terms of inhospital treatment, use differed not significantly between two groups. We found that all 29 patients in the group 1 had severe pneumonia, 18 patients had severe pneumonia. Hospitalization rates, length of hospital stay, and transferred to intensive care unit were found to be statistically significantly higher in the group 1. In the present study, COVID‐19 cases in the large family cluster were shown to have more severe disease and worse clinical course compared with consecutive patients with COVID‐19 presenting to the same time. We believe further studies into potential genetic mechanisms of host susceptibility to COVID‐19 should include such family clusters. 相似文献
36.
Rabia Miray Kisla Ekinci Sibel Balci Dilek Ozcan Bahriye Atmis Atil Bisgin 《European journal of medical genetics》2021,64(9):104262
Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease and usually involves the skin, musculoskeletal system, and kidneys. More than 30 genes have been to monogenic lupus, so far. Monogenic lupus is often characterized by an early-onset, similar family history, and syndromic appearance. Herein we present a pediatric patient with DNASE1L3 deficiency, suffering from both urticarial skin lesions, recurrent hemoptysis, and renal involvement, eventually diagnosed as this rare monogenic lupus.The patient suffered from recurrent urticarial rash and hemoptysis since the age of 15 months of age. He had microscopic hematuria, mild proteinuria, hypocomplementemia, and positive antinuclear antibody, anti-dsDNA, and antineutrophil cytoplasmic antibodies. Renal biopsy yielded immunocomplex glomerulonephritis. Due to early-onset, similar sibling history and consanguineous parents, we suspected monogenic lupus and performed whole-exome sequencing, which further revealed a homozygous T97Ifs*2 mutation (NM_004944.4: c.290_291delCA/p.Thr97Ilefs*2) in DNASE1L3 gene.In conclusion, DNASE1L3 deficiency should be thought when juvenile SLE occurs with early disease-onset, pulmonary hemorrhage, glomerulonephritis, and recurrent urticarial rash along with ANCA positivity. 相似文献
37.
Cakir Karabas Hulya Ozcan Ilknur Erturk Ahmet Faruk Guray Beliz Unsal Gurkan Senel Sukriye Neslihan 《Oral Radiology》2021,37(3):403-411
Oral Radiology - The purpose of this study is to evaluate CBCT images of impacted mandibular canines in detail and to discuss implications for diagnosis and treatment. CBCT images of dental... 相似文献
38.
Isler Sila Cagri Soysal Fatma Ozcan Erkan Saygun N. Isıl Unsal F. Berrin Baris Emre Ilikci Rahsan 《Clinical oral investigations》2021,25(6):3555-3565
Clinical Oral Investigations - The aim of this study was to analyze the mRNA and protein expression of adiponectin, leptin, visfatin, tumor necrosis factor (TNF)-α, and interleukin (IL)-6... 相似文献
39.
Isler Sila Cagri Soysal Fatma Akca Gülcin Bakirarar Batuhan Ozcan Gonen Unsal Berrin 《Odontology / the Society of the Nippon Dental University》2021,109(1):103-113
Odontology - The aim of this trial was to analyze the effect of implant surface decontamination procedures combined with reconstructive surgical treatment (RST) of peri-implantitis on gene... 相似文献
40.