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921.
Briano Di Rezze Mary Law Kevin Eva Nancy Pollock Jan Willem Gorter 《Developmental medicine and child neurology》2013,55(8):737-744
Aim
To increase research rigour and create a plausible way to assess clinical effectiveness, it is necessary to measure the degree to which interventions are delivered as intended (fidelity). Generic fidelity measures enable evaluation of more than one intervention through observation of unique and general characteristics relevant across interventions. This study describes the first generic fidelity measure in paediatrics.Method
Items were constructed from multiple sources to create a general attributes domain and two paediatric cerebral palsy (CP) intervention‐specific domains. After a pre‐testing procedure, raters were trained and videos of clinical interventions were rated to test the measure's psychometric properties.Results
The Paediatric Rehabilitation Observational measure of Fidelity (PROF) consisted of 30 items. Six raters were trained on the PROF and rated 25 videos. Internal consistency (α) and interrater reliability (IRR) for the frequency scale showed the following results: context therapy: α=0.71, IRR=0.75; child therapy: α=0.85, IRR=0.87; and general attributes; α=0.78, IRR=0.82. Quality scale scores across domains demonstrated internal consistency greater than 0.80 and interrater reliability of less than 0.40. Pearson's correlations (r=?0.71, p<0.001) and analyses of variance (p=0.01) validated that each intervention domain was an independent construct.Interpretation
The PROF is reliable and valid for evaluating interventions used for children with CP. Future studies may use the measure's framework, general attributes domain, and procedures to test the psychometric properties of other interventions.922.
923.
Simona Portaro Olimpia Musumeci Vincenzo Rizzo Carmelo Rodolico Mary G. Sweeney Maria Buccafusca Michael G. Hanna Antonio Toscano 《Neuromuscular disorders : NMD》2013,23(1):52-55
A 24-year-old woman complained of a 4-year history of muscle cramps, stiffness of the right lower limb and walking difficulties. After clinical and laboratory investigations, a diagnosis of multiple sclerosis was made. However, her family history revealed that her father and an older sister had lifelong symptoms of impaired muscle relaxation following contraction, improving with physical exercise. Molecular genetic studies in both sisters confirmed the diagnosis of myotonia congenita, due to a c.568GG>TC (Gly190Ser) pathogenic mutation in CLCN1 gene. Occurrence of two different neurological conditions in the same patient, both manifesting with stiffness, is quite unusual and suggests the opportunity of an accurate differential diagnosis. 相似文献
924.
Anne M. Connolly Julaine M. Florence Mary M. Cradock Elizabeth C. Malkus Jeanine R. Schierbecker Catherine A. Siener Charlie O. Wulf Pallavi Anand Paul T. Golumbek Craig M. Zaidman J. Philip Miller Linda P. Lowes Lindsay N. Alfano Laurence Viollet-Callendret Kevin M. Flanigan Jerry R. Mendell Craig M. McDonald Erica Goude Michelle Eagle 《Neuromuscular disorders : NMD》2013,23(7):529-539
Therapeutic trials in Duchenne Muscular Dystrophy (DMD) exclude young boys because traditional outcome measures rely on cooperation. The Bayley III Scales of Infant and Toddler Development (Bayley III) have been validated in developing children and those with developmental disorders but have not been studied in DMD. Expanded Hammersmith Functional Motor Scale (HFMSE) and North Star Ambulatory Assessment (NSAA) may also be useful in this young DMD population. Clinical evaluators from the MDA-DMD Clinical Research Network were trained in these assessment tools. Infants and boys with DMD (n = 24; 1.9 ± 0.7 years) were assessed. The mean Bayley III motor composite score was low (82.8 ± 8; p ? .0001) (normal = 100 ± 15). Mean gross motor and fine motor function scaled scores were low (both p ? .0001). The mean cognitive comprehensive (p = .0002), receptive language (p ? .0001), and expressive language (p = .0001) were also low compared to normal children. Age was negatively associated with Bayley III gross motor (r = ?0.44; p = .02) but not with fine motor, cognitive, or language scores. HFMSE (n = 23) showed a mean score of 31 ± 13. NSAA (n = 18 boys; 2.2 ± 0.4 years) showed a mean score of 12 ± 5. Outcome assessments of young boys with DMD are feasible and in this multicenter study were best demonstrated using the Bayley III. 相似文献
925.
Lisa Pan Matthew T. Keener Stefanie Hassel Mary L. Phillips 《International review of psychiatry (Abingdon, England)》2013,25(4):368-379
Bipolar disorder (BP) is among the top ten most disabling illnesses worldwide. This review includes findings from recent studies employing functional neuroimaging to examine functional abnormalities in neural systems underlying core domains of the psychopathology in BP: emotion processing, emotion regulation and executive control, and common comorbid features of BP, that are relevant to the wide spectrum of BP rather than focused on the more traditional BPI subtype, and that may facilitate future identification of diagnostically-relevant biomarkers of the disorder. In addition, an emerging number of studies are reviewed that demonstrate the use of neuroimaging to elucidate biomarkers whose identification may help to (1) identify at-risk individuals who will subsequently develop the illness to facilitate early intervention, (2) identify targets for treatment and markers of treatment response. The use of newer neuroimaging techniques and potential confounds of psychotropic medication upon neuroimaging findings in BP are also examined. These approaches will help to improve diagnosis and the mental well-being of all individuals with BP. 相似文献
926.
927.
928.
Valproic acid is commonly used in the treatment of both focal and generalized epilepsies and is often well tolerated. There are many reported cases of hyperammonemic encephalopathy and other well-known side effects reported during use of valproic acid either alone or in combination with other antiepileptics. This case report demonstrates valproic acid toxicity in the presence of lacosamide, which has not previously been reported. Full recovery occurred after withdrawal of both valproic acid and lacosamide. 相似文献
929.
Irina Patwardhan W. Alex Mason Mary B. Chmelka Jukka Savolainen Jouko Miettunen Marjo-Riitta Järvelin 《Nordic journal of psychiatry》2013,67(6):340-348
AbstractPurpose: This study examined a developmental model that links affect-regulation difficulties in childhood with three dimensions of alexithymia in adolescence (difficulty identifying feelings, difficulty describing feelings, and externally oriented thinking) and substance use and depression in adulthood, while accounting for cumulative contextual risk in childhood, and testing potential gender moderation.Methods: Multiple group path analyses were conducted using data from the Northern Finland Birth Cohort 1986 (N?=?6963). Analyses used data collected during prenatal/birth, childhood, adolescence, and young adulthood periods.Results: Our examination of early precursors for alexithymia indicated that the associations of affect-regulation problems in childhood with alexithymia were stronger for girls, potentially putting girls with affect-regulation difficulties in childhood at higher risk for developing alexithymia in adolescence. The associations of cumulative contextual risk in childhood with alexithymia, substance use disorder, and depression diagnosis in adulthood were significant for both girls and boys. Our findings in regard to substance use and depression disorders revealed that alexithymia in adolescence predicted depression diagnosis in adulthood, particularly due to a contribution from the alexithymia domain of ‘difficulties identifying feelings.’ However, none of the alexithymia domains was directly associated with substance use disorder in adulthood.Conclusions: Our study contributes to research that links alexithymia with difficulties in affect regulation and cumulative contextual risk in childhood, yielding findings that may be relevant for preventive interventions. 相似文献
930.