86,587 research outputs found
Correction to: Outcomes on safety and efficacy of left atrial appendage occlusion in end stage renal disease patients undergoing dialysis (Journal of Nephrology, (2021), 34, 1, (63-73), 10.1007/s40620-020-00774-5)
No full textThe article Outcomes on safety and efficacy of left atrial appendage occlusion in end stage renal disease patients undergoing dialysis, written by Simonetta Genovesi, Luca Porcu, Giorgio Slaviero, Gavino Casu, Silvio Bertoli, Antonio Sagone, Monique Buskermolen, Federico Pieruzzi, Giovanni Rovaris, Alberto Montoli, Jacopo Oreglia, Emanuela Piccaluga, Giulio Molon, Mario Gaggiotti, Federica Ettori, Achille Gaspardone, Roberto Palumbo, Francesca Viazzi, Marco Breschi, Maurizio Gallieni, Gina Contaldo, Giuseppe D’Angelo, Pierluigi Merella, Fabio Galli, Paola Rebora, Mariagrazia Valsecchi, and Patrizio Mazzone, was originally published electronically on the publisher’s internet portal on 6 June 2020 without open access. With the author(s)’ decision to opt for Open Choice the copyright of the article changed on 10 July 2020 to © The Author(s) 2020 and this article is licensed under a Creative Commons Attribution 4.0 International License (http://creat iveco mmons .org/licen ses/ by/4.0/), which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The original article has been updated
Data set from Bernardini A, Camporeale A, Pieroni M, Pieruzzi F, Figliozzi S, Lusardi P, Spada M, Mignani R, Burlina A, Carubbi F, Battaglia Y, Graziani F, Pica S, Tondi L, Chow K, Boveri S, Olivotto I, Lombardi M. Atrial Dysfunction Assessed by Cardiac Magnetic Resonance as an Early Marker of Fabry Cardiomyopathy. JACC Cardiovasc Imaging. 2020 Oct;13(10):2262-2264. doi: 10.1016/j.jcmg.2020.05.011. Epub 2020 Jun 17. PMID: 32563647.
No full textData set from the article Bernardini A, Camporeale A, Pieroni M, Pieruzzi F, Figliozzi S, Lusardi P, Spada M, Mignani R, Burlina A, Carubbi F, Battaglia Y, Graziani F, Pica S, Tondi L, Chow K, Boveri S, Olivotto I, Lombardi M. Atrial Dysfunction Assessed by Cardiac Magnetic Resonance as an Early Marker of Fabry Cardiomyopathy. JACC Cardiovasc Imaging. 2020 Oct;13(10):2262-2264. doi: 10.1016/j.jcmg.2020.05.011. Epub 2020 Jun 17. PMID: 32563647.
Brief introduction
Anderson-Fabry disease (AFD) cardiomyopathy is characterized by glycosphingolipid (Gb3) storage in all cellular components, with consequent left ventricular hypertrophy (LVH). Gb3 accumulation also involves atrial myocytes (1), ultimately leading to left atrial (LA) enlargement and reduced atrial compliance. Cardiac magnetic resonance (CMR) plays an important role in the assessment of the severity of Fabry cardiomyopathy. CMR feature tracking (CMR-FT) specifically allows the assessment of myocardial strain from cine image
Data set from the article Camporeale A, Pieroni M, Pieruzzi F, Lusardi P, Pica S, Spada M, Mignani R, Burlina A, Bandera F, Guazzi M, Graziani F, Crea F, Greiser A, Boveri S, Ambrogi F, Lombardi M. Predictors of Clinical Evolution in Prehypertrophic Fabry Disease. Circ Cardiovasc Imaging. 2019 Apr;12(4):e008424. doi: 10.1161/CIRCIMAGING.118.008424. PMID: 30943767.
No full textdata set from the article Camporeale A, Pieroni M, Pieruzzi F, Lusardi P, Pica S, Spada M, Mignani R, Burlina A, Bandera F, Guazzi M, Graziani F, Crea F, Greiser A, Boveri S, Ambrogi F, Lombardi M. Predictors of Clinical Evolution in Prehypertrophic Fabry Disease. Circ Cardiovasc Imaging. 2019 Apr;12(4):e008424. doi: 10.1161/CIRCIMAGING.118.008424. PMID: 30943767.
This is the abstract:
Background: In prehypertrophic Fabry disease, low myocardial T1 values, reflecting sphingolipid storage, are associated with early structural and ECG changes. The correlations between T1 values and functional parameters have not been explored. Furthermore, the potential prognostic role of T1 in predicting disease worsening is still unknown.
Methods: ECG, 2D echocardiography, cardiopulmonary test, and cardiac magnetic resonance were performed in 44 Fabry patients without left ventricular hypertrophy (35.7±14.5 years, 68.2% females). After a 12-month follow-up, clinical stability was evaluated using Fabry Stabilization Index.
Results: At baseline, T1 values showed a negative correlation with left ventricular mass ( r=-0.79; P20%) at follow-up. Higher left ventricular wall thickness (odds ratio, 2.61; CI, 1.04-6.57; P=0.04), left atrial volume (odds ratio, 1.24; CI, 1.02-1.51; P=0.03), and lower T1 values (odds ratio, 0.98; CI, 0.96-0.99; P=0.03) at baseline were independently associated with clinical worsening at follow-up.
Conclusions: In prehypertrophic Fabry disease, low T1 values correlate with early electrocardiographic, morphological cardiac changes, and worsening of global disease severity but are not associated with functional abnormalities. The presence of low T1 values is a risk factor for disease worsening, thus representing a potential new tool in prognostic stratification and therapeutic approach
Clinicopathological characteristics of typical and atypical anti-glomerular basement membrane nephritis
No full textAnti-glomerular basement membrane (GBM) antibody disease is a rare pathological condition that mainly involves renal and/or pulmonary parenchyma. It is characterized by the presence of circulating anti-GBM antibodies accompanied by a linear deposition of immunoglobulins (Ig) detected through immunofluorescence (IF) technique and typical signs and symptoms of organ dysfunction, such as rapidly progressive glomerulonephritis (RPGN) and pulmonary hemorrhage (PH). However, recently atypical forms of anti-GBM disease have been described and the presence of overlapping diseases contributed to make its diagnosis challenging. In this review will be discussed the entire spectrum of renal anti-GBM related conditions, focusing the attention on the differences in terms of pathogenesis, diagnosis and therapy of these disparate entities
Area Cardiologica-Advisory Board Plan Multidisciplinare "Diagnosi e Follow-up Malattia di Fabry".Cardiological follow-up in patients with Fabry disease.
No full textFabry disease is a rare tesaurismosis due to a deficit of the lysosomal enzyme activity of alpha-galactosidase, needed for the normal catabolism of globotriaosylceramides (GL3). Fabry cardiac involvement has several clinical manifestations: concentric left ventricular hypertrophy without left ventricular dilation and severe loss of left ventricular systolic function, mitral and aortic valvulopathy, disorders of the atrioventricular conduction or repolarization, and compromised diastolic function. Differentiating Fabry disease from similar conditions is often quite straightforward, e.g., cardiac amyloidosis is often associated with low electrocardiographic voltages, and systemic symptoms are usually associated with hemochromatosis and sarcoidosis. However, sometimes second-level (genetic analysis, alpha-galactosidase levels) or invasive investigations are required, which can include endomyocardial biopsy. Diagnostic imaging techniques have been described, but they lack specificity. Echocardiographic imaging with tissue Doppler analysis and/or strain rate analysis can allow diagnosis of Fabry disease even before left ventricular hypertrophy becomes apparent. This review illustrates the techniques for staging cardiac involvement and damage in Fabry disease and for the long-term follow-up of Fabry patients with or without cardiac involvement. Careful cardiac monitoring is especially important in elderly female carriers, who often develop renal disorders and/or left ventricular hypertrophy as the only manifestations of their late Fabry disease. In some clinical series, Fabry disease was diagnosed in 12% of women with adult-onset hypertrophic cardiomyopathy. Cardiological problems and outcomes of enzyme replacement therapy, associated with or without other cardiological treatments, are also discussed
Variations on the Author
No full text“Variations on the Author” discusses two of Eduardo Coutinho’s recent films (Um Dia na Vida, from 2010, and Últimas Conversas, posthumously released in 2015) and their contribution to the general question of documentary authorship. The director’s filmography is characterized by a consistent yet self-effacing form of authorial self-inscription: Coutinho often features as an interviewer that rather than express opinions propels discourses; an interviewer that is good at listening. This mode of self-inscription characterizes him as an author who is not expressive but who is nonetheless markedly present on the screen. In Um Dia na Vida, however, Coutinho is completely absent form the image, while Últimas Conversas, on the contrary, includes a confessional prologue that moves the director from the margins to the center of his films. This article examines the ways in which these works stand out in the filmography of a director who offers new insights into the notion of cinematic authorship
Analysis of heart period and arterial pressure variability in childhood hypertension : key role of baroreflex impairment
No full textIn adults, initial stages of hypertension are associated with elevated sympathetic drive and significant alterations in indirect autonomic markers. There is growing evidence that children in the highest-pressure percentiles will be more likely to develop hypertension in adulthood, although mechanisms are not understood. We assessed whether computer analysis of RR interval and arterial blood pressure variability could detect early autonomic alterations in childhood hypertension, as assessed by noninvasive time and frequency domain measures of baroreflex regulation. We studied 75 children, subdivided in 3 subgroups of similar age (9.7+/-0.2 years): control subjects, prehypertensive children (ie, children with arterial pressure values >90th and 95th percentile; systolic arterial pressure: 97+/-3/57+/-2, 121+/-5/70+/-1, and 128+/-2/80+/-2 mm Hg, respectively). We observed that hypertensive children demonstrate a significant impairment of the baroreflex as compared with control subjects (index alpha: 20+/-2 and 40+/-4 ms/mm Hg; spontaneous baroreflex slope: 20+/-2 and 37+/-5; ms/mm Hg; P<0.05 in both cases) and reduced RR variance. A similar baroreflex impairment is also observed in children whose arterial pressure falls short of this limit, ie, in the prehypertensive range. In conclusion, hypertensive children display a marked baroreflex impairment. A similar baroreflex impairment is also observed in the prehypertensive state. Baroreflex assessment could furnish additional information in the clinical assessment of pediatric hypertension
Intrafamilial phenotypic variability in four families with Anderson-Fabry disease.
No full textWe analysed the clinical history of 16 hemizygous males affected by Anderson-Fabry Disease, from four families, to verify their intrafamilial phenotypic variability. Seven male patients, ranging from 26 to 61 years of age, died, whereas nine (age range 23-55) are alive. Eleven patients have undergone enzyme replacement therapy (ERT) for a period of 5-10 years. We have found a wide range of intrafamilial phenotypic variability in these families, both in terms of target-organs and severity of the disease. Overall, our findings confirm previous data from the literature showing a high degree of intrafamilial phenotypic variability in patients carrying the same mutation. Furthermore, our results underscore the difficulty in giving accurate prognostic information to patients during genetic counselling, both in terms of rate of disease progression and involvement of different organs, when such prognosis is solely based on the patient's family history
[Newspaper Clipping: Author Claims Evidence of Second JFK Assassin #1]
No full textNewspaper article titled "Author Claims Evidence of Second JFK Assassin." The article states that author Richard J. Whalen concluded "that there is circumstantial evidence to support the theory of a second assassin in the shooting of President John F. Kennedy.
Nuove indicazioni all’impiego del TSH umano ricombinante (rhTSH) e basse attività di 131I nella radioablazione del residuo tiroideo post-chirurgico.
No full textL’ablazione con radioiodio (RRA) in pazienti con carcinoma differenziato della tiroide a rischio basso/intermedio è un argomento di acceso dibattito sia per l’attività di 131I che per la modalità di preparazione [ipotiroidismo vs eutiroidismo con TSH umano ricombinante (rhTSH)]. Recentemente è stato dimostrato che la percentuale ablativa era simile in pazienti preparati con rhTSH o in ipotiroidismo e trattati con alte o basse attività di 131I. È stato inoltre dimostrato che la modalità di preparazione alla RRA non determinava alcuna differenza nello stato finale di malattia a 10 anni dal trattamento, eliminando così ogni esitazione sull’affidabilità della RRA con rhTSH e basse attività di 131I
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