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51.
Atsuko Iwata Takahisa Hirota Koji Konno Takeo Fujimoto Shusuke Sumida Keiji Sato 《Pediatric hematology and oncology》2013,30(7):465-469
A 4-month old girl was diagnosed as having stage IV neuroblastoma of the right adrenal gland. Preoperative chemotherapy was given, followed by local surgical excision. Postoperatively, irradiation of the tumor bed and adjuvant chemotherapy was given for 11 months. Nine years after cessation of chemotherapy, the patient developed left hip-joint pain. Biopsy of the ischium showed chondroblastic osteosarcoma. Limb salvage surgery was performed after preoperative chemotherapy. Postoperatively, adjuvant chemotherapy was given for 14 months. Twenty-two months after treatment for the secondary osteosarcoma, the patient has been remained in disease-free condition without any evidence of relapse. A second osteosarcoma occurring outside the radiation field after treatment for neuroblastoma is quite rare. This unusual case emphasized the need for close monitoring for development of second malignant neoplasms in survivors of neuroblastoma even in the absence of a known predisposing factor, such as radiation therapy. 相似文献
52.
Eiji Wada Mitsuru Fukui Kazuhisa Takahashi Daisaku Takeuchi Hiroshi Hashizume Masahiko Kanamori Noboru Hosono Tsukasa Kanchiku Yuichi Kasai Miho Sekiguchi Shin-ichi Konno Mamoru Kawakami Kazuo Yonenobu 《Journal of orthopaedic science》2019,24(1):57-61
Background
In 1999, the Japanese Orthopaedic Association decided to develop a new Cervical Myelopathy Evaluation Questionnaire (JOACMEQ). The final version of the JOACMEQ, comprising 24 questions and five domains (cervical spine function (CF); upper extremity function (UF); lower extremity function (LF); bladder function (BF); and quality of life (QOL)), was established after three nationwide investigations. The fourth investigation, reported in this paper, was performed to confirm the responsiveness of the questionnaire.Methods
A total of 137 patients with cervical myelopathy were included in the study. Each patient was interviewed twice using the JOACMEQ before and after treatment. At the second interview, the patients self-rated their condition in five domains for “worse,” “somewhat worse,” “no change,” “somewhat better,” or “better,” and these scores were defined as the external assessment rating. The difference of the points in five domains between the first and the second interview was calculated against each external assessment. Based on the results, substantial clinical benefit (SCB) thresholds for the JOACMEQ were determined.Results
The statistically significant median values of the acquired points were 17.5 for CF, 16.0 and 21.0 for UF, 27.0 and 20.5 for LF, 13.0 for BF, and 29.0 for QOL. After consideration of the results, the committee decided that an acquired point ≥20 could be interpreted as representing an SCB threshold for the JOACMEQ.Conclusion
We have concluded that a treatment can be judged to be effective for a patient if 1) The patient give all answers for the questions necessary to calculate the functional score of a domain and an increase of ≥20 points is obtained for that score, or 2) The functional score after treatment is > 90 points even if the answer for the unanswered questions was supposed to be the worst possible choice. 相似文献53.
54.
N Hizawa H Makita Y Nasuhara M Hasegawa K Nagai Y Ito T Betsuyaku S Konno M Nishimura 《The European respiratory journal》2008,32(2):372-378
It was previously reported that the gain-of-function -28 guanine allele of the promoter single nucleotide polymorphism (SNP; cytosine to guanine substitution of nucleotide -28 (-28C>G)) in the CC chemokine ligand 5 gene (CCL5) was associated with susceptibility to late-onset asthma in patients who developed asthma at age > or =40 yrs. The clinical diagnosis of chronic obstructive pulmonary disease (COPD) includes emphysema and small airway disease, and upregulation of CCL5 has been described in the airways of patients with COPD. It was hypothesised that CCL5 has a genetic impact upon the variable expression of emphysema in patients with COPD. Patients with COPD were studied (n = 267). All of the patients underwent pulmonary high-resolution computed tomography (CT), and visual scoring (CT score) was performed to determine emphysema severity. Three SNPs of CCL5 were genotyped, including -403G>A, -28C>G and 375T>C. A significant difference was found in CT score according to CCL5 genotype; the -28G allele was inversely associated with CT score. When the analysis was confined to 180 patients with bronchial reversibility of <15%, even stronger evidence for this association was noted. Functional single nucleotide polymorphisms in the CC chemokine ligand 5 gene were associated with milder emphysema. Together with previous findings, the present study may identify the CC chemokine ligand 5 gene as part of a common pathway in the pathogenesis of late-onset asthma and chronic obstructive pulmonary disease with milder emphysema. 相似文献
55.
Noboru Fujino Masami Shimizu Hidekazu Ino Masato Yamaguchi Toshihiko Yasuda Mitsuru Nagata Tetsuo Konno Hiroshi Mabuchi 《The American journal of cardiology》2002,89(1):29-33
Familial hypertrophic cardiomyopathy (HC) can be caused by mutations in 9 different genes encoding sarcomere proteins expressed in cardiac muscle. To date, only 13 different mutations in the cardiac troponin T (cTnT) gene have been reported to cause HC. Clinical characteristics and prognosis associated with mutations of this gene have not been well characterized owing to the small size and composition of affected families. The aim of this study was to determine the characteristic phenotype of patients with HC caused by a novel cTnT gene mutation, Lys273Glu. Two hundred Japanese probands with HC were screened for mutations in the cTnT gene. The Lys273Glu missense mutation was present in 9 persons from 2 unrelated pedigrees. They exhibited different cardiac morphologies: 1 had a dilated cardiomyopathy-like feature, 7 had left ventricular hypertrophy with normal left ventricular systolic function, and the 6 of them had asymmetric septal hypertrophy. A 1-year-old boy was not evaluated with echocardiography. The mean maximum wall thickness was 18.0 +/- 5.5 mm (range 8 to 24). There were 7 histories of sudden death in 1 of the 2 families. The Lys273Glu substitution in the cTnT gene shows a high degree of penetrance (100% in persons aged >20 years), a high incidence of sudden death, and a partial transition from hypertrophic to dilated cardiomyopathy. Because the location of a mutation appears to influence the development of a phenotype, we suggest that the precise definition of the disease-causing mutation can provide important prognostic information about affected members. 相似文献
56.
Kato S Nakayama K Minoura T Konno M Tajiri H Matsuhisa T Iinuma K;Japanese pediatric Helicobacter study group 《Journal of gastroenterology》2004,39(11):1045-1050
Background As noninvasive tests for Helicobacter pylori infection, the 13C-urea breath test (UBT) and stool antigen test have been widely used. In children, however, there are few studies reporting which test shows superior performance. The purpose of this study was to compare the 13C-UBT and stool antigen test for their accuracy in diagnosing H. pylori infection in children.Methods A total of 123 Japanese children, ages 2 to 17 years (mean, 12 years) who underwent gastric biopsies for H. pylori infection were studied. The diagnoses included gastritis (n = 55), gastric ulcer (n = 5), duodenal ulcer (n = 20), iron-deficiency anemia (n = 7), and other conditions (n = 36). The cutoff value of the 13C-UBT was defined to be 3.5. The stool antigen test was performed using the HpSA enzyme-linked immunosorbent assay (ELISA) (Premier Platinum HpSA). In 16 patients who received eradication therapy, the 13C-UBT and HpSA were repeated 2 months after treatment.Results Based on biopsy tests, 60 children were infected with H. pylori and 63 children were not. For the 13C-UBT, the sensitivity, specificity, and accuracy were 95.0% (95% confidence interval [CI], 86.1%–99.0%), 98.4% (95% CI, 91.5%–100%), and 96.4% (95% CI, 93.6%–99.9%), respectively. For the HpSA, the sensitivity, specificity, and accuracy were 98.3% (95% CI, 90.8%–100%), 98.4% (95% CI, 91.2%–100%), and 98.3% (95% CI, 96.0%–100%), respectively. There were no significant differences between the performance of these two tests. In the assessment of H. pylori eradication, the results of 13C-UBT and HpSA agreed with those of biopsy tests.Conclusions The 13C-UBT and the HpSA are equally accurate for the diagnosis of active H. pylori infection in Japanese children.Kazuie Iinuma, for the Japanese Pediatric Helicobacter study Group 相似文献
57.
The objectives of this study were to understand transition patterns and mechanisms of functional status, and to estimate active life expectancy (ALE) among senior citizens in a small town in Japan. With data drawn from surveys conducted annually from 1998 to 2002 (n=638 at baseline), prevalence and incidence of functional disability in activities of daily living (ADL) and instrumental activities of daily living (IADL) were described and compared between the sexes. Then relationships between potential predictors and functional decline through a 4-year follow-up were examined using logistic regression. Finally, active and disabled life expectancy was estimated by Katz's method. At baseline, 9 and 12% of subjects were dependent in performing ADL and IADL, respectively. Prevalence and incidence rates increased with age. The risk of mortality increased by 2.2-5.0-fold when the subject was functionally dependent at the previous year. Advanced age, difficulty in walking and poor interest were shown to be significant predictors of loss of independence in ADL. Mean durations with disability in ADL and IADL among women were longer than among men by around 1 year. Population-specific preventive care programs considering physical, cognitive and social aspects are needed not only to maximize ALE but also to improve quality of life during survival period with disability especially in old women. 相似文献
58.
Excessive urinary excretion of methionine in mutant mice lacking D-amino-acid oxidase activity 总被引:1,自引:0,他引:1
Thin-layer chromatography and amino acid analysis showed that mutant (ddY/DAO-) mice lacking D-amino-acid oxidase activity excreted about 3.5 times more methionine in urine than did normal (ddY/DAO+) mice. High-performance liquid chromatography using a chiral column showed that approximately 82% of urinary methionine of the ddY/DAO- mice had the D-configuration. Analysis revealed that the mouse diet used contained 0.04% free methionine and that approximately 46% of methionine was the D-isomer. When the ddY/DAO- mice were given a diet containing a low level of supplementary DL-methionine or a diet without D-methionine, they excreted the normal levels of methionine. These results indicate that the ddY/DAO- mice were unable to metabolize D-methionine and excrete it in urine. 相似文献
59.
60.
Masayoshi Yokoyama Atsushi Sakamoto Souji Konno Shigeru Sakakibara 《American heart journal》1974,87(1):15-20
Patients with mitral stenosis usually showed a marked increase in the P negativity following exercise. The P terminal force in Lead V1 in 20 cases with isolated mitral stenosis was ?0.090 mm. sec. before exercise, which changed to ?0.177 mm. sec. following the single Master two-step test.Normal adults never showed such changes on exercise. The phenomenon was considered to be due to the posterior rotation of the P wave vector in the horizontal plane, which was induced by the enlargement of the left atrial wall on exercise. 相似文献