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101.
Yuri A. Zarate Luigi Boccuto Sujata Srikanth Rini Pauly Eylem Ocal Tonya Balmakund Kevin Hinkle Vikki Stefans Gerald B. Schaefer Ronnie Thomas Collins II 《American journal of medical genetics. Part A》2019,179(6):1047-1052
Kosaki overgrowth syndrome is a recently described syndrome characterized by distinctive facial features, brain white matter lesions, and developmental delay. Germline activating heterozygous PDGFRB mutations have been reported in this condition. Systemic connective tissue‐type findings have been described in some individuals. We describe a 19‐year‐old Caucasian female with a history of hydrocephalus, Dandy–Walker malformation, cervical spine arachnoid cyst, progressive scoliosis, and overgrowth. Her physical exam included distinctive craniofacial dysmorphism, as well as soft and hyperextensible skin. Cardiovascular imaging during adolescence revealed saccular aneurysms in both coronary artery systems and subtle tortuosity of the cervical vertebral arteries. Exome sequencing trio analysis identified a de novo previously reported pathogenic variant in PDGFRB, c.1696T>C (p.[Trp566Arg]). Further functional studies included platelet‐derived growth factor cellular metabolic pathway activity that confirmed the variant causes a constitutive activation of the PI3K‐AKT pathway. This is the first report to characterize the activating nature of this PDGFRB variant. We also highlight the connective tissue findings seen in Kosaki overgrowth syndrome and recommend baseline echocardiographic evaluation in all individuals with this condition with particular emphasis on coronary arteries. 相似文献
102.
KM Kanal NJ Hangiandreou AM Sykes HE Eklund PA Araoz JA Leon BJ Erickson 《Journal of digital imaging》2002,14(1):30-37
The aims of this work were to measure the accuracy of one continuous speech recognition product and dependence on the speaker's
gender and status as a native or nonnative English speaker, and evaluate the product's potential for routine use in transcribing
radiology reports. IBM MedSpeak/Radiology software, version 1.1 was evaluated by 6 speakers. Two were nonnative English speakers,
and 3 were men. Each speaker dictated a set of 12 reports. The reports included neurologic and body imaging examinations performed
with 6 different modalities. The dictated and original report texts were compared, and error rates for overall, significant,
and subtle significant errors were computed. Error rate dependence on modality, native English speaker status, and gender
were evaluated by performing ttests. The overall error rate was 10.3 +/- 3.3%. No difference in accuracy between men and women
was found; however, significant differences were seen for overall and significant errors when comparing native and nonnative
English speakers (P = .009 and P = .008, respectively). The speech recognition software is approximately 90% accurate, and
while practical implementation issues (rather than accuracy) currently limit routine use of this product throughout a radiology
practice, application in niche areas such as the emergency room currently is being pursued. This methodology provides a convenient
way to compare the initial accuracy of different speech recognition products, and changes in accuracy over time, in a detailed
and sensitive manner. 相似文献
103.
104.
Canfield MC; Tamarappoo BK; Moses AM; Verkman AS; Holtzman EJ 《Human molecular genetics》1997,6(11):1865-1871
Congenital nephrogenic diabetes insipidus (NDI) is a rare disease caused
most often by mutations in the vasopressin V2 receptor (AVPR2). We studied
a family which included a female patient with NDI with symptoms dating from
infancy. The patient responded to large doses of desmopressin (dDAVP) which
decreased urine volume from 10 to 4 I/day. Neither the parents nor the
three sisters were polyuric. The patient was found to be a compound
heterozygote for two novel recessive point mutations in the aquaporin-2
(AQP2) gene: L22V in exon 1 and C181W in exon 3. Residue Cys181 in AQP2 is
the site for inhibition of water permeation by mercurial compounds and is
located near to the NPA motif conserved in all aquaporins. Osmotic water
permeability (Pf) in Xenopus oocytes injected with cRNA encoding C181W-AQP2
was not increased over water control, while expression of L22V cRNA
increased the Pf to approximately 60% of that for wild-type AQP2.
Co-injection of the mutant cRNAs with the wild-type cRNA did not affect the
function of the wild-type AQP2. Immunolocalization of AQP2-transfected CHO
cells showed that the C181W mutant had an endoplasmic reticulum-like
intracellular distribution, whereas L22V and wild-type AQP2 showed endosome
and plasma membrane staining. Water permeability assays showed a high Pf in
cells expressing wild-type and L22V AQP2. This study indicates that AQP2
mutations can confer partially responsive NDI.
相似文献
105.
Debelenko LV; Brambilla E; Agarwal SK; Swalwell JI; Kester MB; Lubensky IA; Zhuang Z; Guru SC; Manickam P; Olufemi SE; Chandrasekharappa SC; Crabtree JS; Kim YS; Heppner C; Burns AL; Spiegel AM; Marx SJ; Liotta LA; Collins FS; Travis WD; Emmert-Buck MR 《Human molecular genetics》1997,6(13):2285-2290
Lung carcinoids occur sporadically and rarely in association with multiple
endocrine neoplasia type 1 (MEN1). There are no well defined genetic
abnormalities known to occur in these tumors. We studied 11 sporadic lung
carcinoids for loss of heterozygosity (LOH) at the locus of the MEN1 gene
on chromosome 11q13, and for mutations of the MEN1 gene using dideoxy
fingerprinting. Additionally, a lung carcinoid from a MEN1 patient was
studied. In four of 11 (36%) sporadic tumors, both copies of the MEN1 gene
were inactivated. All four tumors showed the presence of a MEN1 gene
mutation and loss of the other allele. Observed mutations included a 1 bp
insertion, a 1 bp deletion, a 13 bp deletion and a single nucleotide
substitution affecting a donor splice site. Each mutation predicts
truncation or potentially complete loss of menin. The remaining seven
tumors showed neither the presence of a MEN1 gene mutation nor 11q13 LOH.
The tumor from the MEN1 patient showed LOH at chromosome 11q13 and a
complex germline MEN1 gene mutation. The data implicate the MEN1 gene in
the pathogenesis of sporadic lung carcinoids, representing the first
defined genetic alteration in these tumors.
相似文献
106.
Maša Davidović Nadine Zielonke Iris Lansdorp-Vogelaar Nereo Segnan Harry J. de Koning Eveline AM. Heijnsdijk 《Value in health》2021,24(3):353-360
ObjectivesTo quantify the impact of mammography-based screening on the quality of life, disability-adjusted life years (DALYs) averted or quality-adjusted life years (QALYs) gained can be used. We aimed to assess whether the use of DALYs averted or QALYs gained will lead to different cost-effective screening strategies.MethodsUsing the microsimulation model MISCAN, we simulated different breast cancer screening strategies varying in starting age (starting at 45, 47, and 50 years), stopping age (stopping at 69, 72, and 74 years), and frequency (annual [A], biennial [B], combination of both [A + B], and triennial [T]). In total, we defined 24 different breast cancer screening strategies, including no screening as a reference strategy. We calculated incremental cost-effectiveness ratios (ICERs) and compared which strategies were on the efficiency frontiers for DALYs and QALYs.ResultsBreast cancer screening averted between 46.00 and 105.58 DALYs and gained between 28.69 and 64.50 QALYs per 1000 women. For DALYs there were 5 strategies on the efficiency frontier (T50-69, T50-74, T45-74, B45-74, and A45-74). The same strategies plus one (B45-72) were on the efficiency frontier for QALYs.ConclusionsUsing DALYs averted instead of QALYs gained to assess the effects on quality of life from breast cancer screening in the Dutch population yields differences in ICERs, but almost the same strategies were on the efficiency frontiers. Whether the choice in outcome measure leads to a difference in optimal policy depends on the cost-effectiveness threshold. 相似文献
107.
Schaefer Jürgen R. von Hirschhausen Eckart 《Bundesgesundheitsblatt, Gesundheitsforschung, Gesundheitsschutz》2021,64(1):21-27
Bundesgesundheitsblatt - Gesundheitsforschung - Gesundheitsschutz - Menschen mit komplexen und seltenen Erkrankungen haben es in unserem Gesundheitssystem oft schwer. Bis zur Diagnosefindung kann... 相似文献
108.
109.
The authors have previously described an extradural transmaxillary approach to the anterior compartment of the cavernous sinus. In an effort to expand the surgical access to that area without necessitating a craniotomy or wide transfacial dissection, they present a modification of the transmaxillary approach to the sellar region and cavernous sinus. Methods: The approach was developed on 12 fresh and 12 embalmed cadaveric specimen, and 2 dry skulls. The initial sublabial incision is followed by a maxillotomy to expose the course of the infraorbital nerve (terminal branch of maxillary branch of the trigeminal nerve) on the roof of the maxillary sinus. The route of the infraorbital nerve is traced to the pterygopalatine fossa as a guide to the foramen rotundum. Superomedial drilling of the foramen rotundum is then performed to reveal the contents of the superior orbital fissure. After the nerves are safely identified in the superior orbital fissure, medial enlargement of the window into the cavernous sinus is made possible by drilling the lateral and posterior wall and septum of the sphenoid sinus. Results: The combined transmaxillary transsphenoidal approach offers an excellent exposure of the sellar and infrasellar region. The approach offers clear visualization of the ipsilateral loop of the carotid artery, the pituitary fossa, and the cranial nerves of the ipsilateral cavernous sinus. Mean operative reach is 38 mm from the posterior wall of the maxillary sinus to the ipsilateral carotid loop and 56 mm to the contralateral loop. The width of the operative window is 26 mm at the base within the cavernous sinus. Conclusion: The model offers a minimally invasive approach that avoids the need for craniotomy or violating the nasal cavity. It may be safely employed to access vascular as well as invasive lesions of the sellar and infrasellar region. The approach offers excellent visualization of the ipsilateral intracavernous carotid artery with both proximal and distal control, as well as cranial nerves III, IV, VI, V2, the hypophyseal region, and the medial aspect of the contralateral cavernous sinus. 相似文献
110.
With increasing pressure from third-party payers to assess client outcomes, clinical programs want to know how to implement outcome systems. This article focuses on practical and logistic questions involved in implementing an outcome assessment system in ambulatory behavioral healthcare settings. Study questions addressed outcome systems in general and the use of the Behavior and Symptom Identification Scale (BASIS-32) and the Short Form Health Status Profile (SF-36) in particular. General questions focused on obtaining provider buy-in, client consent and confidentiality, data collection methods, sampling, time points, maximizing client participation, clinical utility of outcome data, and resources needed for outcome assessment. Measure-specific questions focused on client acceptability of the instruments and applicability of measures to diverse populations. The article suggests several strategies for enhancing outcome assessment efforts and concludes that there remains a need for further understanding of ways to maximize the utility and value of outcome measurement. 相似文献