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71.
TF Leung WC Tsoi CK Li KW Chik MMK Shing PMP Yuen 《Acta paediatrica (Oslo, Norway : 1992)》1998,87(6):705-777
We describe a 15-y-old girl with Fechtner-like syndrome, who is the first Chinese reported to have this rare syndrome. She presented with left homonymous hemianopia and neuroimaging revealed haemorrhage in both parietal and occipital lobes. Peripheral blood smear showed macrothrombocytopenia and intracytoplasmic inclusion bodies inside leucocytes. Thrombocytopenia and proteinuria responded to intravenous immunoglobulin and pulsed methylprednisolone. This case illustrates that life-threatening haemorrhage can occur in patients with Fechtner syndrome. Although there was no effective treatment reported in the literature, high dose steroid and immunoglobulin seemed to be useful in our patient. Our patient also had nephritic-nephrotic syndrome with renal insufficiency, which is unusual in adolescent female patients. 相似文献
72.
Susumu Kusunoki Atsuro Chiba Seiji Hitoshi Hajime Takizawa Ichiro Kanazawa 《Muscle & nerve》1995,18(4):409-413
Four of 82 patients with Guillain-Barré syndrome (GBS) and 1 of 12 with multifocal motor neuropathy (MMN), who previously had had Mycoplasma pneumoniae infections, had serum antibody to galactocerebroside (Gal-C). Two patients with GBS without mycoplasma infection also had anti-Gal-C antibody, whereas none of the normal or the disease controls had it. As Gal-C is a major glycolipid antigen in myelin, anti-Gal-C antibody may function in the pathogenesis of autoimmune demyelinative neuropathies. Mycoplasma pneumoniae appears to be an important preceding infectious agent in autoimmune neuropathies with anti-Gal-C antibody. © 1995 John Wiley & Sons, Inc. 相似文献
73.
Familial Sneddon's syndrome 总被引:4,自引:0,他引:4
Alexander Lossos Tamir Ben-Hur Ziva Ben-Nariah Claes Enk Moshe Gomori Dov Soffer 《Journal of neurology》1995,242(3):164-168
We report the familial occurrence and apparent autosomal dominant inheritance of Sneddon's syndrome with variable clinical expression. The proband, a 40-year-old woman, presented with livedo reticularis and progressive neurological deterioration following a stroke. The diagnosis was confirmed by cerebral angiogram and skin biopsy, both showing the characteristic findings. Two of the patient's sisters were reported to have been similarly affected in the past. Her mother, two additional siblings and five of her seven children exhibited various vasospastic skin phenomena. Familial aggregation of this disorder may be common and a genetic basis may be involved in its pathogenesis. 相似文献
74.
75.
Diagnosis and treatment of thoracic outlet syndrome 总被引:2,自引:0,他引:2
Masataka Abe Tadashi Shimamura Jun Nishida Katsuaki Ichinohe 《Journal of orthopaedic science》1997,2(2):119-127
Patients who develop symptoms of thoracic outlet syndrome (TOS) have a predisposing anatomic abnormality. In most patients
with TOS, the symptoms are caused by entrapment of the brachial plexus and they do not arise from compression of the subclavian
artery, as was previously thought. The tests advocated for diagnosing this common syndrome (i.e., evaluating the positional
compression of the artery when the arms are raised, the neck is turned, or the shoulders are braced) cannot accurately diagnose
this syndrome. There are two reasons for this. The symptoms of TOS are not related to the compression of the artery in the
outlet in 98% of patients, and 75% of normal individuals without symptoms show diminished radial pulse on various provocation
tests. We employed four timed provocation tests (minute tests) to diagnose TOS: the timed Morley test, timed Wright test,
timed Eden test, and elevated arm stress exercise, all of which are very sensitive. In normal individuals without symptoms,
20% experience transitional symptoms such as slight pain and tiredness, on these tests indicating a subclinical state. TOS
is treated by keeping the thoracic outlet wide, this being done either conservatively or surgically. In 1993 and 1994, we
conservatively treated 418 of 422 patients with TOS by means of active exercise, a brace, and by block therapy. These measures
did not reduce the symptoms in 23 of these patients, so surgical treatment was indicated. In the remaining 4 of the 422 patients,
conservative treatment was not indicated and surgery was performed directly. All the patients showed significant clinical
improvement of varying degree.
Presented at the 69th Annual Meeting of the Japanese Orthopaedic Association, Tokyo, April 12, 1996 相似文献
76.
P. Peeters J. Sennesael H. De Raeve M. De Waele D. Verbeelen 《Transplant international》1997,10(6):471-474
Lymphoma in immunocompromised transplant patients is a feared cause of morbidity and mortality. Superimposed on the lymphoma
and the transplantation immunosuppression is a rare condition: hemophagocytic syndrome (HS). HS is characterized by fever,
hepatosplenomegaly and lymphadenopathy, skin rashes, jaundice, coagulopathy, and phagocytosis of blood elements with pancytopenia.
Here we describe a rare but fatal case of a kidney transplant patient who developed T-cell lymphoma and HS, without evidence
of EBV replication. A short review of the diagnosis, treatment, and prognosis of HS is given.
Received: 4 March 1997 Received after revision: 6 June 1997 Accepted: 30 June 1997 相似文献
77.
小儿肾脏疾病血,尿内皮素的变化 总被引:9,自引:0,他引:9
目的研究小儿肾脏疾病血、尿内皮素(PET、UET)的水平及其相互关系。方法采用同位素放免方法检测了肾病综合征(NS),肾小球肾炎(GN),肾功能衰竭(RF)共72例患儿血及尿中ET,血心钠素(ANP)水平。结果NS,GN,RF三组的PET及UET值明显高于对照组,尤其RF组(P<005,P<0.01)。ANP值在GN组和RF组明显高于对照组(P<001)。8例ARF患儿恢复期血ET水平下降,6例CRF患儿虽经治疗,但血ET水平不降或上升。结论ET在小儿肾脏疾病发病机理及病情进展中可能起重要作用,其值高低与病情严重程度及预后有关。 相似文献
78.
探讨老年人微小病变性(MC)特发性肾病综合征(PNS)的临床特征及预后。方法 观察成人MC性PNS118例,其中老年患者18例,占15.2%。结果 发现老年患者NS的临床表现与成年组相似;但血尿发病率较低,22%比38%;高血压和肾功能不全的发病率明显增高,38.9%比16%和33.3%比19%(P<0.01)。老年组对激素治疗的敏感性略差;但NS复发率明显减少,11.1%比43%。结论 尽管NS的复发和对激素依赖使治疗有一定困难,但激素、细胞毒性药物和环孢素A对于消除蛋白尿,延缓肾功能不全是有助的。 相似文献
79.
成人原发性肾病综合征血容量与肾素,醛固酮,心钠素的关系 总被引:2,自引:0,他引:2
探讨成人原发性肾病综合征(NS)血容量与肾素、醛固酮及心钠素的关系。方法 应用~(113)mInCl标记转铁蛋白稀释法测定血容量,放免法测定血浆激素水平。对水肿期NS28例、正常26例及其中NS缓解期随访18例进行检测。结果 (1)水肿期NS血容量与正常组无差别,血浆肾素活性(PRA)、血管紧张素Ⅱ(ATⅡ)、醛固酮(Ald)、心钠素(ANP)水平均较正常组高,白蛋白、各激素水平与血容量无显著相关;(2)缓解期与水肿期比较,总的血容量无差异,Ald、ANP显著降低,PRA、ATⅡ则无明显差异;(3)Ald与24小时尿排钠(UNaV)显著负相关。结论 Ald和ANP是NS钠排泄的主要调节因子。 相似文献
80.
Kodama Hideya; Fukuda Jun; Karube Hiroko; Matsui Toshihiko; Shimizu Yasushi; Tanaka Toshinobu 《Human reproduction (Oxford, England)》1995,10(8):1962-1967
This study was aimed at assessing the outcome of in-vitro fertilization(IVF) and embryo transfer in patients with polycystic ovariansyndrome (PCOS). The results of IVF and embryo transfer in PCOSpatients (PCOS group, 78 cycles of 26 patients) were comparedwith those of a control group (423 cycles in 202 patients withoutmale factor; age and ovarian stimulation protocol were matched).Although the pregnancy rate per transfer was not different inthe two groups of patients (25 versus 34%, PCOS versus controlgroup), the PCOS group had a significantly lower pregnancy rateper follicle aspiration (19 versus 31%, P < 0.05). A notableresult was a significantly higher incidence of embryo transfercancellations in the PCOS group (22 versus 8%, P < 0.01),which resulted from unpredictable failure of either oocyte recoveryor fertilization. The incidence of unexplained complete failureof fertilization was significantly higher in the PCOS group(18 versus 5%, P < 0.01). These results may reflect a reducedquality of the oocytes in the PCOS group, and there was a subgroupof PCOS patients who repeatedly produced poor results of treatment.Although the ovarian stimulation regimen best suited to PCOSpatients remains to be determined, special care should be takenduring ovarian stimulation, especially when the PCOS patientshad experienced unexplained failure of oocyte recovery or fertilizationin the previous treatment cycle(s). 相似文献