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Amir H. Taghinia Joseph Upton Cameron C. Trenor Ahmad I. Alomari Anna P. Lillis Raja Shaikh Patricia E. Burrows Steven J. Fishman 《Journal of pediatric surgery》2019,54(3):562-568
Background
Central conducting lymphatic anomalies (CCLA) may cause chylous leaks and protein-losing enteropathy (PLE) owing to dysfunction of the central lymphatic channels. Most of the treatment strategies for these conditions are palliative and provide transient improvement.Methods
We treated 14 patients with intractable chylous leak and/or PLE using a novel technique of lymphaticovenous bypass of the terminal portion of the thoracic duct. Chylous leaks occurred in multiple different anatomic sites. All patients had CCLA and failure of thoracic duct emptying demonstrated by preoperative intranodal lymphangiography.Results
Five patients had complete resolution of symptoms, and two patients had partial improvement. There were no major complications. Of 5 patients with PLE, only one improved after lymphaticovenous bypass. Repeat traditional lymphangiography was performed in 4 patients who did not improve, demonstrating patency of the bypass in all cases with persistent sluggish drainage. One patient had repeat MR lymphangiography that did not show the thoracic duct well.Conclusions
Bypass of the terminal thoracic duct is a novel procedure that offers improvement and a chance of cure for some patients with devastating manifestations of CCLA who lack other effective therapeutic options.Level of evidence
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Tan WH Baris HN Burrows PE Robson CD Alomari AI Mulliken JB Fishman SJ Irons MB 《Journal of medical genetics》2007,44(9):594-602
Background
Mutations in the PTEN gene cause two disorders that predispose to cancer, Bannayan–Riley–Ruvalcaba and Cowden syndromes. Some patients with a PTEN mutation have only macrocephaly and autism, but they may still be at risk for neoplasms. Vascular anomalies occur in patients with a PTEN mutation, but they have not been systematically studied or precisely defined.Method
We analysed the clinical and radiological features of the vascular anomalies in 26 patients with PTEN mutations who were either seen or had their medical records reviewed at Children''s Hospital Boston.Results
All 23 patients who had their head circumference measured were macrocephalic, and all 13 male patients who were fully examined had penile freckling. Vascular anomalies were found in 14/26 (54%) of patients: 8/14 (57%) had multiple lesions and 11/13 (85%) who had cross‐sectional imaging had intramuscular vascular lesions. Radiographic studies showed that 12/14 (86%) were fast‐flow vascular anomalies, and angiography typically showed focal segmental dilatation of draining veins. Excessive ectopic fat in the vascular anomalies was present in 11/12 (92%) of patients on CT or MRI. Intracranial developmental venous anomalies (DVAs) were found in 8/9 (89%) of patients who had brain MRI with contrast.Conclusions
Vascular anomalies in patients with a PTEN mutation are typically multifocal intramuscular combinations of fast‐flow channels and ectopic fat. Cerebral DVAs are very common. PTEN mutational analysis should be considered for all macrocephalic patients with fast‐flow vascular anomalies or multiple intracranial DVAs. 相似文献7.
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This study aimed to explore the outcomes of active participation in an action research project on building the research capacity of clinical nurses. In this qualitative research study, six registered nurses volunteered to participate in the action research team. None of the nurses reported having any prior research experience. This study was part of a larger three‐phase project. The nurses were required to reflect on the data about their medication practice of phase 1, develop and implement a bundle of interventions in phase 2 to improve medication safety, and evaluate the effectiveness of these interventions in phase 3. We report the nurses' participation in Action Research during phase 2. Meeting minutes and six semistructured interviews were thematically analyzed. The results showed that after receiving support to enhance their research skills from the research team, the nurses were empowered to perform and lead clinical nursing research project. Nurses were able to take ownership of the research process and outcomes and were then able to translate their new research knowledge and skills into their clinical practice by building their own research capacity. 相似文献
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PIK3CA‐related overgrowth spectrum (PROS): Diagnostic and testing eligibility criteria,differential diagnosis,and evaluation
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Kim M. Keppler‐Noreuil Jonathan J. Rios Victoria E.R. Parker Robert K. Semple Marjorie J. Lindhurst Julie C. Sapp Ahmad Alomari Marybeth Ezaki William Dobyns Leslie G. Biesecker 《American journal of medical genetics. Part A》2015,167(2):287-295
Somatic activating mutations in the phosphatidylinositol‐3‐kinase/AKT/mTOR pathway underlie heterogeneous segmental overgrowth phenotypes. Because of the extreme differences among patients, we sought to characterize the phenotypic spectrum associated with different genotypes and mutation burdens, including a better understanding of associated complications and natural history. Historically, the clinical diagnoses in patients with PIK3CA activating mutations have included Fibroadipose hyperplasia or Overgrowth (FAO), Hemihyperplasia Multiple Lipomatosis (HHML), Congenital Lipomatous Overgrowth, Vascular Malformations, Epidermal Nevi, Scoliosis/Skeletal and Spinal (CLOVES) syndrome, macrodactyly, Fibroadipose Infiltrating Lipomatosis, and the related megalencephaly syndromes, Megalencephaly‐Capillary Malformation (MCAP or M‐CM) and Dysplastic Megalencephaly (DMEG). A workshop was convened at the National Institutes of Health (NIH) to discuss and develop a consensus document regarding diagnosis and treatment of patients with PIK3CA‐associated somatic overgrowth disorders. Participants in the workshop included a group of researchers from several institutions who have been studying these disorders and have published their findings, as well as representatives from patient‐advocacy and support groups. The umbrella term of “PIK3CA‐Related Overgrowth Spectrum (PROS)” was agreed upon to encompass both the known and emerging clinical entities associated with somatic PIK3CA mutations including, macrodactyly, FAO, HHML, CLOVES, and related megalencephaly conditions. Key clinical diagnostic features and criteria for testing were proposed, and testing approaches summarized. Preliminary recommendations for a uniform approach to assessment of overgrowth and molecular diagnostic testing were determined. Future areas to address include the surgical management of overgrowth tissue and vascular anomalies, the optimal approach to thrombosis risk, and the testing of potential pharmacologic therapies. © 2014 The Authors. American Journal of Medical Genetics Part A published by Wiley Periodicals, Inc. 相似文献
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