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This report describes four siblings affected with familial intrahepatic cholestasis detected in early infancy. In the two male siblings, biliary cirrhosis and fatal hepatocellular carcinoma later developed, whereas the female siblings have had persistent hepatomegaly and recurrent episodes of cholestasis. Sequential biopsies show that this rare disorder of unknown etiology must be added to the many causes of giant cell transformation of the liver in infancy. Its oncogenic risk, particularly in males, has not been generally appreciated.  相似文献   
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Our laboratory has characterized a population of stromal cells obtained from adipose tissue termed processed lipoaspirate cells (PLAs). PLAs, like bone-marrow derived mesenchymal stem cells (BM-MSCs), have the capacity to differentiate along the adipogenic, osteogenic, chondrogenic, and myogenic lineages, In order to better characterize these two multi-lineage populations, we examined the surface phenotype of both bone marrow and adipose tissue-derived cells from five patients undergoing surgery. PLA and BM-MSC cells were isolated, subcultivated, and evaluated for cell surface marker expression using flow cytometry. PLA and BM-MSC cells both expressed CD13, CD29, CD44, CD90, CD105, SH-3, and STRO-1. Differences in expression were noted for cell adhesion molecules CD49d (Integrin alpha4), CD54 (ICAM-1), CD34, and CD106 (VCAM-1). While markedly similar, the surface phenotypes of PLA and BM-MSC cells are distinct for several cell adhesion molecules implicated in hematopoietic stem cell homing, mobilization, and proliferation.  相似文献   
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The COVID-19 pandemic has significantly impacted daily activities worldwide. University students may have experienced substantial changes in daily living as a result of restrictions on university attendance. The return to normalcy may take a long time, and understanding the influence that shifts in daily routines have had on the lifestyles of university students may inform approaches to support overall well-being. We analyzed changes in the lifestyles of students enrolled at a health sciences university during the COVID-19 pandemic. This longitudinal study took place at the Faculty of Medicine and Nursing in the University of the Basque Country in Spain, and the final sample consisted of 113 nursing students, 109 medical students, and 45 physiotherapy students. Our results demonstrate changes in lifestyles of university students during the pandemic. MedDiet adherence scores and the percentage of students with high adherence increased during the pandemic. This increase was due to the increased consumption of vegetables and nuts. In terms of physical activity, the practice of moderate and intense physical activity was maintained. These results provide important information for both public health authorities and educational institutions to guide strategies to maintain the well-being of students and enhance opportunities for young adults to lead a healthy lifestyle.  相似文献   
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OBJECTIVE: To evaluate the appropriateness of the use of total hip joint replacements. DESIGN: Observational study of consecutive patients with a diagnosis of hip osteoarthritis and who had undergone total hip arthroplasty over a 1-year period from seven hospitals. MAIN MEASURES: The appropriateness of the use of hip replacement was judged by explicit criteria developed by a panel of experts using RAND methodology. The length of hospital stay during the admission and complications were recorded 6 months post-operatively. Patients were also surveyed 6 months after discharge to determine whether they believed they had recovered or their satisfaction with the intervention. Appropriateness results of this study were compared with a previous study performed with the same criteria 4 years previously. RESULTS: In total, 784 patients participated in the study. Indications for surgery were considered necessary in 52.2% of cases, appropriate in 21.3%, uncertain in 21.4%, and inappropriate in 5.1%. Differences were found in the rates of appropriateness exclusively from one hospital. At 6 months after discharge, differences between centres were found for the proportion of patients that reported they had recovered from surgery (range 57.7-24.8%) and in the length of hospital stay during admission (range 10-16 days). Improvement in the appropriateness rates were found for all participant hospitals during both periods. CONCLUSIONS: We identified a low percentage of inappropriate indications and differences in some outcomes between centres. Compared with previously, there has been improvement in the use of this technique, although both periods are not methodologically comparable.  相似文献   
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Extended cervical mediastinoscopy in the staging of bronchogenic carcinoma   总被引:3,自引:0,他引:3  
BACKGROUND: Extended cervical mediastinoscopy (ECM) is a technique in the staging of bronchogenic carcinoma described first by Ginsberg in 1984. To update our experience we have prospectively evaluated our results on 106 patients who underwent the technique from 1985 to 1998. METHODS: The ECM technique is performed once the operability of the patient has been evaluated, according to the computed tomography findings. The intervention is carried out at the same time as a standard cervical mediastinoscopy through the same incision following the same technique as previously published. The ECM is considered positive when metastatic nodes or tumor involvement directly in the paraaortic or subaortic regions is detected and confirmed histologically. Negative cases of ECM and positive cases of standard cervical mediastinoscopy are excluded from this study. A false-negative ECM is defined as the presence of infiltrated adenopathies at the paraortic level detected on postoperative histologic study. RESULTS: We had performed ECM in 106 patients, and a total of 13 were subsequently excluded for the reasons stated above. Of the remaining cases, 26 were positive, 61 negative and 6 had false-negative results with no false-positive results. Sensitivity was 81.2%, specificity 100%, accuracy 93.3%, positive predictive value 100%, and negative predictive value 91%. There were no complications with the technique. CONCLUSIONS: We conclude that ECM is a useful technique for staging bronchogenic carcinoma that allows samples to be taken from paraortic and subaortic regions with minimally invasive techniques.  相似文献   
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Diarrhetic shellfish poisoning (DSP) toxins of algal origin are frequent contaminants of coastal waters and seafood. The potential risk for human health due to the continuous presence of these toxins in food has not been clearly established. We have used cerebellar primary cultures to investigate the effects of the DSP toxin dinophysistoxin-2 (DTX-2) on central nervous system neurons and glial cells. Exposure to DTX-2 produced neurotoxicity at concentrations starting at 2.5 nM, characterized first by disintegration of neurites and later by cell death. DTX-2-induced neurodegeneration required long exposures (at least 20 h), involved DNA fragmentation and condensation and fragmentation of chromatin, typical hallmarks of apoptosis, and required the synthesis of new proteins. The concentration that reduced by 50% the maximum neuronal survival after 24 h exposure to DTX-2 (EC50(24)) was approximately 8 nM. Morphology and viability of glial cells remained unaffected up to at least 15 nM DTX-2. Higher concentrations of the toxin caused strong shrinkage of glial cell bodies and retraction of processes, and a significant reduction of glial cell viability. Glial toxicity by DTX-2 involved typical apoptotic condensation and fragmentation of chromatin. Compared to neurons, the effect on glial cells was a much shorter process, and extensive glial degeneration and death occurred after 7 h exposure to DTX-2 (EC50(7) approximately 50 nM; EC50(24) approximately 30 nM). Although further experiments are needed to confirm these toxic actions in vivo, our in vitro data suggest that chronic exposure to amounts of DSP toxins below the current safety regulatory limits may represent a risk for human health that should be taken into consideration.  相似文献   
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The objective of this study was to identify the social factors that explain the differences in knowledge with regards to HIV/AIDS among immigrants in the Basque Country (Spain). We conducted a cross-sectional study based on information obtained in the Basque Health Survey for 754 immigrants from: 86 China, 368 Latin America, 237 the Maghreb and 74 Senegal. Odds ratios (95% CI) were calculated from logistic regression models to measure the degree of association between inadequate knowledge regarding transmission, prevention and places where HIV testing is offered, and the independent variables. We found that this inadequate knowledge is associated with place of birth, sex, a lower level of education, immigration status, difficulties in understanding Spanish, and not receiving advice about AIDS in primary care. These findings indicate that initiatives must be developed to promote equity in the provision of healthcare through clinical guidelines, including details of the specific needs of different groups of immigrants and considering gender issues.  相似文献   
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