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91.
Shelley A. Klemm Richard D. Gordon Terry J. Tunny Wendy L. Finn 《Clinical and experimental pharmacology & physiology》1990,17(3):191-195
1. Plasma potassium and chloride concentrations were raised and plasma renin activity, aldosterone, bicarbonate and arterial pH were reduced in two brothers with the syndrome of hypertension and hyperkalaemia with normal glomerular filtration rate (Gordon's syndrome), on unrestricted or moderately restricted sodium diets. 2. These abnormalities were corrected in both patients within 10 days of severe sodium restriction. 3. Pressor sensitivity to cold and angiotensin II decreased on low sodium diet, associated with a fall in blood pressure. 4. Increasing distal tubular sodium delivery by infusion of normal saline increased fractional excretion of potassium when aldosterone had been stimulated by severely restricted sodium diet, but not when aldosterone levels were low on unrestricted sodium diet. 5. These findings are consistent with excessive sodium reabsorption as the primary renal lesion in Gordon's syndrome, leading to volume expansion and suppression of renin and aldosterone. Severe dietary sodium restriction leading to volume contraction, by stimulating renin and aldosterone and promoting kaliuresis, corrects the abnormalities. 相似文献
92.
对我科1989-1990年中收治的婴儿肝炎综合征63例的临床表现、合并症、治疗等方面进行了分析。笔者认为婴儿肝炎综合征为一组临床症候群。病因诊断有其重要性。腹部B超检查有助于肝炎和胆道闭锁的鉴别,可作为常规检查方法。并认为用肾上腺皮质激素治疗有效。 相似文献
93.
Bilateral ulnar agenesis is a rare abnormality. A total of 36 cases are analyzed: 35 of these are documented in the literature and 1 stillborn male is presented in this study. Most patients had one of the three conditions: Al-Awadi/Raas-Rothschild syndrome, syndrome of ulnar aplasia with split hand/split foot deformity, or the Brachmann—de Lange syndrome. Fifty percent of all cases with bilateral ulnar agenesis were associated with lower limb defects and these cases, for the most part, also belonged to the aforementioned syndromes. Nonskeletal, internal organ malformations were identified in 34% of all patients. Nine patients presented with isolated bilateral ulnar agenesis. The Al-Awadi/Raas-Rothschild syndrome and the split hand/split foot deformity are heritable disorders. There was no evidence for genetic etiology in most of the other cases. Bilateral ulnar agenesis in our fetus was part of the Brachmann—de Lange syndrome with associated cardiac defect, diaphragmatic hernia, and umbilical artery agenesis. 相似文献
94.
目的:探讨新生儿双胎输血综合征的临床表现及其合并症等问题。方法:对过去10年间收治的29对双胎输血综合征患儿资料进行回顾性分析。结果:双胎输血综合征患儿有一般双胎儿的临床共性,且双胎输血综合征患儿中受血儿出现病理临床表现及合并症较供血儿多且严重。结论:双胎输血综合征对受血儿危害更大,故临床上一旦确诊为双胎输血综合征,应对受血儿进行严密监护,若出现临床表现,并发生红细胞增多症,应积极采取治疗措施,以减少严重并发症及后遗症的发生。 相似文献
95.
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
96.
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
97.
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
98.
99.
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
100.
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