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81.
目的:总结一例急性早幼粒细胞白血病(acute promyelocytic leukemia,APL)诱导化疗后合并弥漫性肺泡出血(diffuse alveolar hemorrhage,DAH)的诊治体会,提高对APL并发DAH的认识。方法:报道一例APL患者经维A酸(all-trans retinoic acid,ATRA)联合去甲氧柔红霉素化疗后并发DAH的症状和体征、实验室检查结果、治疗措施。结果:该患者在ATRA化疗后出现发热、咳嗽、咯血、呼吸困难、双肺湿性啰音、低氧血症,X线胸片示弥漫性浸润影,诊断DAH,立即给予激素、高流量吸氧及强有力的抗感染治疗后病情好转。结论:DAH为恶性血液病化疗后少见却易致命的并发症,早期诊断、及时激素治疗可有效提高患者生存率,改善预后。 相似文献
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83.
Sakae Homma Masahito Ebina Kazuyoshi Kuwano Hisatsugu Goto Fumikazu Sakai Susumu Sakamoto Takeshi Johkoh Keishi Sugino Teruo Tachibana Yasahiro Terasaki Yasuhiko Nishioka Koichi Hagiwara Naozumi Hashimoto Yoshinori Hasegawa Akira Hebisawa 《Respiratory investigation》2021,59(1):8-33
This manual has been compiled by a joint production committee with the Diffuse Lung Disease Assembly of the Japanese Respiratory Society (JRS) to provide a practical manual for the epidemiology, diagnosis, and treatment of intractable diffuse pulmonary diseases. The contents are based upon the results of research into these diseases by the Diffuse Pulmonary Diseases Study Group (principal researcher: Sakae Homma) supported by the FY2014–FY2016 Health and Labor Sciences Research Grant on Intractable Diseases.This manual focuses on: 1) pulmonary alveolar microlithiasis, 2) bronchiolitis obliterans, and 3) Hermansky-Pudlak Syndrome with interstitial pneumonia. As these are rare/intractable diffuse lung diseases (2 and 3 were first recognized as specified intractable diseases in 2015), there have not been sufficient epidemiological studies made, and there has been little progress in formulating diagnostic criteria and severity scales; however, the results of Japan's first surveys and research into such details are presented herein. In addition, the manual provides treatment guidance and actual cases for each disease, aiming to assist in the establishment of future modalities.The manual was produced with the goal of enabling clinicians specialized in respiratory apparatus to handle these diseases in clinical settings and of further advancing future research and treatment. 相似文献
84.
Alexander Schwickert MD Karl-Heinz Seeger MD Rebecca C. Rancourt PhD Wolfgang Henrich MD 《Journal of clinical ultrasound : JCU》2019,47(6):366-368
We report a case of a prenatally detected hemangioma of the umbilical cord as an early sign of diffuse neonatal hemangiomatosis (DNH). The newborn was diagnosed with multiple hemangiomas in the liver, intestines, skin, and brain. Prenatal ultrasound findings, neonatal appearance of the hemangiomas, and the associated complications are illustrated. Interdisciplinary investigations as well as operative and systemic treatment approaches proved to be challenging. This case illustrates how prenatal ultrasound with color Doppler facilitates the early diagnosis of DNH and can help through the early referral to specialized centers for appropriate treatment. 相似文献
85.
Wojciech Dabrowski Todd T. Schlegel Jaroslaw Wosko Radoslaw Rola Ziemowit Rzecki Manu L.N.G. Malbrain Andrzej Jaroszynski 《Journal of electrocardiology》2018,51(3):499-507
Introduction
Traumatic brain injury (TBI) affects cardiac electrical function, and several extra-cerebral factors, including intra-abdominal pressure (IAP), might further modulate this brain-heart interaction. The purpose of this study was to investigate the impact of TBI, and of increased IAP during TBI, on cardiac electrical function as measured by vectorcardiographic (VCG) variables.Methods
Survival, IAP and changes in VCG variables including spatial QRS-T angle and QTc interval were measured in consecutive adult patients with either isolated TBI (iTBI), or with TBI accompanied by polytrauma to the abdomen and/or limbs (pTBI). For all patients, observations were performed just after the admission to the ICU (baseline) and at 24, 48, 72 and 96 h after admission.Results
74 patients aged 45 ± 18 were studied. 44 were treated for iTBI and 30 for pTBI. In all patients, spatial QRS-T angle and QTc interval increased after TBI (p < 0.001), relatively more so in patients with pTBI. Compared to survivors, non-survivors also ultimately had greater widening of the spatial QRS-T angle (p < 0.001), most notably just before foraminal herniation. Wider spatial QRS-T angle and longer QTc interval were also noted in patients with IAP > 12 mmHg (p < 0.001), and with right compared to left hemispheric injury (p < 0.001). ST segment level at the J point decreased 24 and 48 h after TBI in leads I, II, III, aVR, aVF, V1, V2, V3 and V6, and increased in lead V1, especially in non-survivors.Conclusions
Spatial QRS-T angle and QTc interval increase after TBI. If foraminal herniation complicates TBI, further widening of the spatial QRS-T angle typically precedes it, followed by notable narrowing thereafter. Increased IAP also intensifies TBI-associated increases in spatial QRS-T angle and QTc interval. 相似文献86.
Hamadani M Benson DM Lin TS Porcu P Blum KA Devine SM 《European journal of haematology》2008,81(6):425-431
The transformation of follicular lymphoma (FL) to high-grade histology occurs in up to 70% of patients. The role of hematopoietic stem cell transplantation (HSCT) in transformed FL is poorly defined. Twenty-four FL patients with histologically confirmed transformation to diffuse large B-cell lymphoma underwent unpurged autologous HSCT at our institution. Their median age was 56 yr. The median number of prior chemotherapies was 2 (range 1-6). Thirteen patients had residual nodal disease measuring more than 2 cm and four patients had bulky disease at the time of HSCT. Six patients had refractory disease at transplantation. At a median follow-up of 38 months, 3-yr progression-free survival following autologous HSCT was 40%. The 3-yr overall survival was 52%. The cumulative incidence of relapse and non-relapse mortality rate was 41% and 25%, respectively. 相似文献
87.
88.
G. Pouessel C. Thumerelle V. Nève T. Santangelo S. Flammarion I. Pruvot I. Tillie-Leblond A. Deschildre 《La Revue de médecine interne / fondée ... par la Société nationale francaise de médecine interne》2014
Juvenile dermatomyositis is the leading cause of chronic idiopathic inflammatory myopathy of auto-immune origin in children. Lung involvement in inflammatory myopathies is well described in adults, involving mostly interstitial lung disease, aspiration pneumonia and alveolar hypoventilation. We propose to describe its specificities in children. Pulmonary involvement may be asymptomatic and therefore must be systematically screened for. In case of clinical or functional respiratory abnormality, a chest computed tomographic (CT) scan is necessary. In children, a decrease of respiratory muscle strength seems common and should be systematically and specifically searched for by non-invasive and reproducible tests (sniff test). Interstitial lung disease usually associates restrictive functional defect, impairment of carbon monoxide diffusion and interstitial lung disease on CT scan. As in adults, the first-line treatment of juvenile dermatomyositis is based on corticosteroids. Corticosteroid resistant forms require corticosteroid bolus or adjuvant immunosuppressive drugs (methotrexate or cyclosporine). There is no consensus in pediatrics for the treatment of diffuse interstitial lung disease. Complications of treatment, including prolonged steroid therapy, are frequent and therefore a careful assessment of the treatments risk-benefit ratio is necessary, especially in growing children. 相似文献
89.
90.
Stephen D. Smith Brian G. Till Mazyar S. Shadman Ryan C. Lynch Andrew J. Cowan Qian V. Wu Jenna Voutsinas Heather A. Rasmussen Katherine Blue Chaitra S. Ujjani Andrei Shustov Ryan D. Cassaday Jonathan R. Fromm Ajay K. Gopal 《British journal of haematology》2020,189(6):1119-1126
Tumor programmed death-ligand 1 (PD-L1) expression in diffuse large B-cell lymphoma (DLBCL) is associated with inferior outcomes. The first-line immunologically-replete setting may be an opportune time for PD-1 inhibition. We evaluated pembrolizumab in combination with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) in untreated patients with DLBCL. Eligible patients were age 18 or older, had adequate organ function, and had DLBCL requiring full-course therapy. Patients received pembrolizumab 200 mg/cycle with R-CHOP, primarily to assess toxicity. Response assessment utilized standard criteria, and PD-L1 staining was performed at a validated central laboratory. Among 30 patients, toxicity was comparable to standard R-CHOP but with two grade ≥3 immune related adverse events (rash, pneumonitis). The overall and complete response rate was 90% and 77%. With 25·5 months of median follow-up, 2-year progression-free survival (PFS) is 83%. PD-L1 expression was associated with non-GCB subtype, and improved PFS and survival. Pembrolizumab can safely be added to R-CHOP, and is associated with a high CR rate and 2-year PFS. Improved PFS with PR-CHOP in PD-L1 expressing tumors contradicts historical data in R-CHOP treated patients, supporting evaluation of PD-L1 as a biomarker to identify DLBCL patients who may benefit from this first-line strategy. 相似文献