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排序方式: 共有56条查询结果,搜索用时 15 毫秒
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Fabi Marianna Andreozzi Laura Frabboni Ilaria Dormi Ada Corinaldesi Elena Lami Francesca Cicero Cristina Tchana Bertrand Francavilla Rosa Sprocati Monica Bigucci Barbara Balsamo Claudia Valin Paola Sogno Di Fazzio Giorgia Iughetti Lorenzo Valletta Enrico Marchetti Federico Donti Andrea Lanari Marcello 《Clinical rheumatology》2021,40(4):1507-1514
Clinical Rheumatology - Kawasaki disease (KD) is the most frequent cause of acquired heart disease in children in high-income countries because of coronary artery involvement. Risk factors for... 相似文献
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This report describes the multicenter experience of the Tachylog antitachycardia pacemaker (Siemens-Elema) in the treatment of drug-refractory, recurrent supraventricular arrhythmias. The device has been implanted in 63 patients (mean age 47 years). The arrhythmias were atrial tachycardia in 4 patients (6%), atrioventricular nodal reentry tachycardia in 23 patients (37%), circus movement tachycardia via an overt bypass tract in 17 patients (27%) and via a concealed bypass tract in 19 patients (30%). The mean follow-up period was 30 months. In 28 patients (44%) arrhythmia control was achieved with the pacemaker therapy alone. In 31 patients (49%) drug therapy had to be reintroduced to obtain control of recurrent arrhythmias. Four patients (6%) were definite nonresponders. In 4 patients (6%) the pulse generator was explanted either because it was not tolerated or because of tachycardia-sensing failure. No syncope or death was observed during follow-up. Thus, antitachycardia pacing gives satisfactory results in selected patients with drug-refractory supraventricular tachycardias. 相似文献
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N Valin F Antoun C Choua?d M Renard B Dautzenberg V Lalande B Ayache P Morin W Sougakoff J M Thiolet C Truffot-Pernot V Jarlier B Decludt 《The international journal of tuberculosis and lung disease》2005,9(5):528-533
SETTING: An overcrowded 362-bed migrants' shelter in Paris, France. OBJECTIVES: To investigate an outbreak of tuberculosis (TB), to identify a common source of contamination and to prevent further transmission. METHODS: The outbreak was identified by radiographic screening and an active search for undeclared hospital treated cases, completed by strain phenotyping and a search for contact cases. RESULTS: Between October 2001 and October 2002, 56 cases of active TB were identified, 30 by radiological screening and 20 by contacting neighbouring hospitals. All cases involved men, with a median age of 30 years. Pulmonary involvement was present in 54% of cases, and nine patients were sputum smear-positive. Thirty-four of the 37 phenotyped strains clustered together. CONCLUSION: The grouping of the cases in time and place, the large number of cases with early-stage disease and the identical RFLP banding patterns of most of the isolates indicate that this outbreak results from transmission that occurred in France. This report underlines the need for public health departments in industrialised countries to maintain effective anti-tuberculosis control programmes. 相似文献
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Edson Donizetti Verri Gabriel Pdua da Silva Evandro Marianetti Fioco Nayara Soares da Silva Saulo Csar Valin Fabrin Cesar Augusto Bueno Zanella Camila Roberta Garrefa Milton Faria Júnior Selma Sissere Jaime Eduardo Cecilio Hallak Marcelo Palinkas Thais Cristina Chaves Simone Cecilio Hallak Regalo 《Journal of oral rehabilitation》2019,46(10):912-919
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Pierre De Rossi Robert J. Andrew Timothy F. Musial Virginie Buggia‐Prevot Guilian Xu Moorthi Ponnusamy Han Ly Sofia V. Krause Richard C. Rice Valentine de lEstoile Tess Valin Someya Salem Florin Despa David R. Borchelt Vytas P. Bindokas Daniel A. Nicholson Gopal Thinakaran 《Brain pathology (Zurich, Switzerland)》2019,29(4):485-501
Bridging integrator 1 (BIN1) is the most significant late‐onset Alzheimer’s disease (AD) susceptibility locus identified via genome‐wide association studies. BIN1 is an adaptor protein that regulates membrane dynamics in the context of endocytosis and membrane remodeling. An increase in BIN1 expression and changes in the relative levels of alternatively spliced BIN1 isoforms have been reported in the brains of patients with AD. BIN1 can bind to Tau, and an increase in BIN1 expression correlates with Tau pathology. In contrast, the loss of BIN1 expression in cultured cells elevates Aβ production and Tau propagation by insfluencing endocytosis and recycling. Here, we show that BIN1 accumulates adjacent to amyloid deposits in vivo. We found an increase in insoluble BIN1 and a striking accrual of BIN1 within and near amyloid deposits in the brains of multiple transgenic models of AD. The peri‐deposit aberrant BIN1 localization was conspicuously different from the accumulation of APP and BACE1 within dystrophic neurites. Although BIN1 is highly expressed in mature oligodendrocytes, BIN1 association with amyloid deposits occurred in the absence of the accretion of other oligodendrocyte or myelin proteins. Finally, super‐resolution microscopy and immunogold electron microscopy analyses highlight the presence of BIN1 in proximity to amyloid fibrils at the edges of amyloid deposits. These results reveal the aberrant accumulation of BIN1 is a feature associated with AD amyloid pathology. Our findings suggest a potential role for BIN1 in extracellular Aβ deposition in vivo that is distinct from its well‐characterized function as an adaptor protein in endocytosis and membrane remodeling. 相似文献
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Brellier F Valin A Chevallier-Lagente O Gorry P Avril MF Magnaldo T 《The British journal of dermatology》2008,159(2):445-452
Background Gorlin syndrome, or naevoid basal cell carcinoma syndrome (NBCCS), is an autosomal dominant disorder associated with mutations in the PTCH1 gene, which encodes the receptor of SONIC HEDGEHOG. In addition to developmental abnormalities, patients with NBCCS are prone to basal cell carcinoma (BCC), the most frequent type of nonmelanoma skin cancer in humans.
Objectives As ultraviolet (UV) exposure plays a prominent role in the development of sporadic BCC, we aimed to determine whether primary NBCCS skin cells exhibit differential responses to UV exposure compared with wild-type (WT) skin cells.
Methods Primary fibroblast and keratinocyte strains were isolated from nonlesional skin biopsies of 10 patients with characteristic NBCCS traits. After identification of PTCH1 mutations, capacities of NBCCS cells to repair UV-induced DNA lesions and to survive after UV irradiation, as well as p53 responses, were compared with those of WT skin cells.
Results The c1763insG PTCH1 mutation is described for the first time. DNA repair and cell survival analyses following UV irradiation revealed no obvious differences between responses of NBCCS and WT fibroblasts and keratinocytes. However, p53 accumulation after UV irradiation was abnormally persistent in all NBCCS primary keratinocyte strains compared with WT keratinocytes.
Conclusions Our observations that NBCCS cells harbour normal DNA repair and survival capacities following UV irradiation better explain that BCC proneness of patients with NBCCS does not solely concern body areas exposed to sunlight and suggest rather that it might be due to cell cycle alterations. 相似文献
Objectives As ultraviolet (UV) exposure plays a prominent role in the development of sporadic BCC, we aimed to determine whether primary NBCCS skin cells exhibit differential responses to UV exposure compared with wild-type (WT) skin cells.
Methods Primary fibroblast and keratinocyte strains were isolated from nonlesional skin biopsies of 10 patients with characteristic NBCCS traits. After identification of PTCH1 mutations, capacities of NBCCS cells to repair UV-induced DNA lesions and to survive after UV irradiation, as well as p53 responses, were compared with those of WT skin cells.
Results The c1763insG PTCH1 mutation is described for the first time. DNA repair and cell survival analyses following UV irradiation revealed no obvious differences between responses of NBCCS and WT fibroblasts and keratinocytes. However, p53 accumulation after UV irradiation was abnormally persistent in all NBCCS primary keratinocyte strains compared with WT keratinocytes.
Conclusions Our observations that NBCCS cells harbour normal DNA repair and survival capacities following UV irradiation better explain that BCC proneness of patients with NBCCS does not solely concern body areas exposed to sunlight and suggest rather that it might be due to cell cycle alterations. 相似文献
8.
Laurent Camous Nadia Valin José Luis Lopez Zaragoza Edward Bourry Eric Caumes Gilbert Deray Hassane Izzedine 《Nephrology, dialysis, transplantation》2008,23(1):393-395
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