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Eran Maman David M. Steinberg Batia Stark Shai Izraeli Shlomo Wientroub 《Journal of children's orthopaedics》2007,1(1):63-68
Purpose Studies on musculoskeletal manifestations (MSM) of childhood acute lymphoblastic leukemia (ALL) have yielded variable findings
with regard to their clinical impact. We investigated the significance for differential diagnosis, treatment and outcome of
musculoskeletal complaints as presenting symptoms of ALL, and their correlation with leukemia immunophenotypes, for which
data is lacking.
Methods Data on 783 children in the national study for childhood ALL between 1984 and 2003 were reviewed retrospectively. Statistical
analysis examined possible relationships between MSM at the time of diagnosis and demographic and clinical data, biological
features of leukemia (peripheral blood counts, immunophenotype and main cytogenetic aberration), response to initial prednisone
treatment, and outcome.
Results Of 765 children with data on orthopaedic complaints, 240 presented with MSM (31.4%). Among these children, B cell precursor
(BCP) was much more common (209/576, 36.3%) than T cell ALL (25/176, 14.2%). Patients with MSM had lower white blood cell
counts (WBC) (median of 9 vs. 20 × 109/L, P < 0.001) and percentage of blast cells in the peripheral blood at diagnosis compared to those without (median of 27 vs. 53%,
P < 0.001). Hepatomegaly and splenomegaly were less common in MSM group (67 vs. 53% <3 cm, P < 0.001, and 63 vs. 50% <3 cm, P < 0.001, respectively). Poor response to initial treatment with prednisone was recorded in 7.1% of patients with MSM versus
11.5% of those without (P = 0.086). The analysis revealed no independent effect of MSM on event-free survival (EFS), after correcting for differences
in EFS related to immunophenotype or initial WBC.
Conclusions MSM occur mostly in children with BCP ALL who present with less involvement of extramedullary organs, low peripheral blood
blasts and white blood cells counts. These findings highlight the importance of including ALL in the differential diagnosis
of MSM even in the presence of an apparently normal peripheral blood count. Our study also suggests that MSM are caused by
leukemic cells with enhanced biological propensity to remain relatively confined within the intramedullary bone-marrow space. 相似文献
5.
We report the case of a patient with delayed methotrexate (MTX)-induced leukoencephalopathy who showed a marked improvement both in clinical and neuroimaging findings after a high-dose of the active form of folinic acid (leucovorin) treatment. The patient developed progressive affective impairment accompanied by headache, nausea and vomiting after treatment with MTX during the chemotherapy for acute lymphoblastic leukemia, and diagnosed as delayed type MTX-induced leukoencephalopathy. After an intravenous injection of high-dose folinic acid (total 1920 mg), neurological deficits and white matter changes dramatically improved in a few weeks. Although delayed MTX-induced leukoencephalopathy may cause irreversible brain damage, an early treatment with high dose leucovorin may thus facilitate the marked improvement of clinical findings and white matter abnormalities. 相似文献
6.
选择分别位于bcr/abl嵌合基因的Mbcrl第二外显子和abl的第二外显子上的两条引物,对18例慢粒白血病及2例急淋白血病标本进行逆转录PCR(RT-PCR)检测。结果:在18例包括ph(+)和ph(-)不同病期的慢粒白血病患者血中均检出bcr/abl嵌合基因的表达,其中14例为K-28型表达,1例为L-6型表达,3例为K-28型,L-6型同时表达;2例急淋白血病标本中1例为K-28型表达。研究结果表明,1.bcr/abl嵌合基因是慢粒白血病的一个重要分子生物学特征;2.ph(+)和ph(-)慢粒白血病的分子生物学基础相同,即均有bcr/abl嵌合基因;3.至少部分急淋白血病与慢粒白血病有相同的分子生物学基础;4.bcr的断裂点位置可能有一定的临床意义,但有待进一步研究;5.同时表达L-6型和K-28型的情况多见于急变区,此现象提示体内可能同时有两类不同嵌合的白血病细胞或白血病急变期bcr出现新的重排。 相似文献
7.
研究了10例正常人和29例急性淋巴细胞性白血病(急淋)患者外周血糖皮质激素受体高、低亲和力结合位,或(GCRH、GCRL),利用Ru38486对GCRL进行封闭,对部分患者GCRH、GCRL在激素联合化疗前后水平的变化进行了动态观察。结果表明,正常对照组GCRH、GCRL分别为4608±1889位点/细胞和135238±88509位点/细胞,两者相关良好。急淋患者GCRH、GCRL分别为6052±3888位点/细胞和126405±102133位点/细胞,经过糖皮质激素药物联合化疗后,其水平分别为3616±1962位点/细胞和143597±112289位点/细胞,GCRH下降明显(P<0.01),下降率为40.3%;而GCRL化疗前后差异不显著(P>0.05)。提示GCRL在介导糖皮质激素联合化疗疗效的维持中起重要的作用。 相似文献
8.
Salim H. Khalil Mohammed H. Qari John M. Jackson Robert Haywood Pyle Hassan El-Solh Abdallah Al-Nasser 《Leukemia research》1994,18(12):881-883
Geographical variations in the incidence of disease are of considerable theoretical and practical importance. It has been claimed that the distribution of acute lymphoblastic leukemia (ALL) phenotypes in Saudi Arabia is different from that recorded in the Western literature. One hundred and twelve (112) patients under 15 years of age, diagnosed as ALL between January 1992 and May 1994 had immunophenotypes performed on their blast cells. Common ALL (cALL) together with pre-B-ALL, formed 86.5% of the total; B-cell 3%, T-cell 6% and null cell 4.5%. These figures are not significantly different from the Western literature. A previous claim from this institution in 1990, that both null and B-cell ALL were significantly increased compared with elsewhere, is not supported by the present figures. Age and sex distribution, and FAB classification, L1 77%, L2 20% and L3 3%, were also of the same order as described elsewhere and, in particular, there was no increase in the frequency of L3 subtype. 相似文献
9.
对40例中国人急性淋巴细胞白血病(急淋)进行了细胞遗传学的研究,发现正常核型为40%,染色体数量异常占42.5%,染色体结构异常为17.5%,特异染色体异常包括有t(9;22)(q34;q11),t(4;11)(q21;q23)和t(8;14)(q24;q32)及环状染色体等.分析了2例急淋患者初发和复发时核型的变化,显示复发时有附加异常。研究认为细胞形态学,免疫表型和细胞遗传学的联合分析(MIC)有助于急淋的诊断和分型。此外,细胞遗传学检查对于急淋白血病的预后具有重要意义。 相似文献
10.
Hirokazu Taniguchi Kenjiro Hamamoto Takahiro Nagano Yuji Kishimoto Takashi Kimura Hideki Fujitake Kojiro Yasunaga 《Medical Electron Microscopy》1993,26(1):13-18
Ultrastructural myeloperoxidase (MPO) activity and myeloid-associated antigen (MyAg) expression were investigated in 12 adult
patients with acute lymphoblastic leukemia (ALL). Ultrastructural MPO was detected by 3 different methods. Immunophenotyping
was performed by flow cytometry, using a series of monoclonal antibodies. Ultrastructural MPO-positive blast cells were detected
in 6 patients (50%). In 5 of these 6 patients, the methods detecting both MPO and platelet peroxidase (PPO) activities found
MPO-positive blast cells more frequently than those detecting MPO activity alone. In 2 patients (17%), at least one kind of
MyAg was positive. Ultrastructural MPO activity was detected more frequently than MyAg expression in ALL patients. This method
of detecting PPO and MPO is recommended for detection of ultrastructural MPO-positive ALL. 相似文献