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71.
Cystic kidney diseases are clinically and genetically heterogeneous. The most important entities are autosomal-dominant and autosomal-recessive polycystic kidney diseases. The proteins encoded by the involved genes are referred to as cystoproteins, which are located predominantly in the primary cilia. Primary cilia play an important role in cyst formation. Inherited polycystic kidney diseases belong to the increasing number of reported ciliopathies, including several syndromic entities. An exact diagnosis is the basis for medical care and genetic counselling; thus, the diagnostic algorithm should include clinical, ultrasonographic and morphological features of the underlying kidney disease, knowledge about further features and family history. Molecular genetic testing may contribute important information towards a definite diagnosis. 相似文献
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Chen-Xu M Topless R McKinney C Merriman ME Phipps-Green A Dalbeth N Gow PJ Harrison AA Highton J Jones PB Nissen M Smith MD van Rij A Jones GT Rodriguez-Rodriguez L Fernandez-Gutierrez B Teruel M Balsa A Pascual-Salcedo D Ortiz AM Gonzalez-Gay MA Steer S Maehlen M Lie B Wordsworth BP Stamp LK Martin J Merriman TR 《Annals of the rheumatic diseases》2012,71(1):155-157
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Ovandir Bazan Jayme Pinto Ortiz Francisco Ubaldo Vieira Junior Reinaldo Wilson Vieira Nilson Antunes Fabio Bittencourt Dutra Tabacow Eduardo Tavares Costa Orlando Petrucci Junior 《Brazilian Journal Of Cardiovascular Surgery》2013,28(4):462-469
Introduction
In vitro hydrodynamic characterization of prosthetic heart valves provides important information regarding their operation, especially if performed by noninvasive techniques of anemometry. Once velocity profiles for each valve are provided, it is possible to compare them in terms of hydrodynamic performance. In this first experimental study using laser doppler anemometry with mechanical valves, the simulations were performed at a steady flow workbench.Objective
To compare unidimensional velocity profiles at the central plane of two bi-leaflet aortic prosthesis from St. Jude (AGN 21 - 751 and 21 AJ - 501 models) exposed to a steady flow regime, on four distinct sections, three downstream and one upstream.Methods
To provide similar conditions for the flow through each prosthesis by a steady flow workbench (water, flow rate of 17L/min. ) and, for the same sections and sweeps, to obtain the velocity profiles of each heart valve by unidimensional measurements.Results
It was found that higher velocities correspond to the prosthesis with smaller inner diameter and instabilities of flow are larger as the section of interest is closer to the valve. Regions of recirculation, stagnation of flow, low pressure, and flow peak velocities were also found.Conclusions
Considering the hydrodynamic aspect and for every section measured, it could be concluded that the prosthesis model AGN 21 - 751 (RegentTM) is superior to the 21 AJ - 501 model (Master Series). Based on the results, future studies can choose to focus on specific regions of the these valves. 相似文献76.
Eizmendi I Almendral J Hadid C Ortiz M 《Journal of cardiovascular electrophysiology》2012,23(3):325-329
We describe the case of a 61-year-old woman who underwent successful catheter cryoablation of a symptomatic Hisian ectopy. Diagnosis was based on features of the HV interval assessed from a bipolar recording during mapping. The location of the arrhythmic focus was identified using simultaneous unipolar and bipolar recordings of the His electrogram. This case report highlights the use of 2 new criteria for the diagnosis and mapping of Hisian ectopy, and the successful use of cryothermia for the ablation of extrasystoles arising from the His bundle. 相似文献
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Influence of Tricuspid Bioprosthetic Mitral Valve Orientation Regarding the Flow Field Inside the Left Ventricle: In Vitro Hydrodynamic Characterization Based on 2D PIV Measurements 下载免费PDF全文
Ovandir Bazan Jayme P. Ortiz Newton K. Fukumasu Antonio L. Pacifico Jurandir I. Yanagihara 《Artificial organs》2016,40(2):175-179
The flow patterns of a prosthetic heart valve in the aortic or mitral position can change according to its type and orientation. This work describes the use of 2D particle image velocimetry (PIV) applied to the in vitro flow fields characterization inside the upper part of a left ventricular model at various heart rates and as a function of two orientations of stented tricuspid mitral bioprostheses. In the ventricular model, each mitral bioprosthesis (27 and 31 mm diameter) was installed in two orientations, rotated by 180°, while the aortic bileaflet mechanical valve (27 mm diameter) remained in a fixed orientation. The results (N = 50) showed changes in the intraventricular flow fields according to the mitral bioprostheses positioning. Also, changes in the aortic upstream velocity profiles were noticed as a function of mitral orientations. 相似文献
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Carlos Morales-Uribe Ana Ramírez Tatiana Suarez-Poveda Margarita Ortiz Alvaro Sanabria 《Emergency radiology》2016,23(5):421-431
This study was conducted in order to define the diagnostic performance of CT angiography for vascular injuries compared with angiography in patients with neck trauma. CT angiography is the cornerstone of diagnosis for hemodynamically stable patients with wounds suspicious of vascular trauma in the limbs, chest, or abdomen. Available evidence for the use of CT angiography in neck vascular trauma comes from small case series and few randomized controlled trials, and high-quality information does not exist regarding its performance. A protocol using the recommendations of the Cochrane Collaboration was designed. A systematic search of diagnostic studies without limits on language or time was carried out to December 2014. Studies including patients with neck trauma with retrospective or prospective data collection that assessed CT angiography compared with other methods were selected. Methodological quality was assessed using the QUADAS-2 tool. A hierarchical model ROC curve and a bivariate random effects model were used for the pooled analysis. Sixteen studies were selected and reviewed, and nine studies with 693 patients were included in this review. The overall sensitivity was 97 % (95 % CI 0.77–1.00; I 2?=?65.7 % (41.4–90.0)), while the overall specificity was 99 % (95 % CI 0.93–1.00; I 2?=?0). The hierarchic ROC curve showed an area under the curve of 0.99. Publication bias was not identified in this study. CT angiography can be stated as the gold standard for diagnosing vascular injuries in hemodynamically stable patients with neck trauma. 相似文献