An imbalance of TNF system activity has been reported in patients with myotonic dystrophy type 1 (DM1). Nevertheless, the question whether TNF-α action is directly implicated in the pathogenesis of DM1 or is a simple marker of disease activity is still open. Therefore, the present study was aimed to investigate serum tumor necrosis factor (TNF)-α, interleukin (IL)-1β, and IL-6 levels in association with the disease stage, cytosine-thymine-guanine (CTG) expansion and cardiac function of 56 patients with DM1 (40±14 years) and 28 healthy controls (42±12 years). All subjects were submitted to resting electrocardiogram (EKG), Signal-averaged EKG (SA-EKG), and M-mode/2-D echocardiography. TNF-α levels were higher in patients compared to controls (p<0.0003) and were associated to disease stage (p<0.02). Significant correlation were observed between TNF and CTG expansion (p<0.005) or PQ intervals (p<0.0005). Ventricular late potentials (VLPs) occurred in 54% of cases. In these patients, TNF-α levels were higher compared to those without VLPs (p<0.05). We may conclude that TNF-α levels might represent and adjunctive criterion for disease staging in patients with myotonic dystrophy type 1, and that elevated TNF levels in DM1 may lead to cardiac fibrosis affecting diastolic function, conduction, and automaticity. © 2002 Elsevier Science B.V. All rights reserved.

Tumor necrosis factor-alpha and myocardial function in patients with myotonic dystrophy type 1 / Mammarella, Antonio; Ferroni, Patrizia; Michele, Paradiso; Francesca, Martini; Paoletti, Vincenzo; Stefania, Morino; Antonini, Giovanni; Gazzaniga, Pierpaolo; Musca, Antonino; Basili, Stefania. - In: JOURNAL OF THE NEUROLOGICAL SCIENCES. - ISSN 0022-510X. - STAMPA. - 201:1-2(2002), pp. 59-64. [10.1016/s0022-510x(02)00193-4]

Tumor necrosis factor-alpha and myocardial function in patients with myotonic dystrophy type 1

MAMMARELLA, Antonio;FERRONI, Patrizia;PAOLETTI, Vincenzo;ANTONINI, Giovanni;GAZZANIGA, Pierpaolo;MUSCA, Antonino;BASILI, Stefania
2002

Abstract

An imbalance of TNF system activity has been reported in patients with myotonic dystrophy type 1 (DM1). Nevertheless, the question whether TNF-α action is directly implicated in the pathogenesis of DM1 or is a simple marker of disease activity is still open. Therefore, the present study was aimed to investigate serum tumor necrosis factor (TNF)-α, interleukin (IL)-1β, and IL-6 levels in association with the disease stage, cytosine-thymine-guanine (CTG) expansion and cardiac function of 56 patients with DM1 (40±14 years) and 28 healthy controls (42±12 years). All subjects were submitted to resting electrocardiogram (EKG), Signal-averaged EKG (SA-EKG), and M-mode/2-D echocardiography. TNF-α levels were higher in patients compared to controls (p<0.0003) and were associated to disease stage (p<0.02). Significant correlation were observed between TNF and CTG expansion (p<0.005) or PQ intervals (p<0.0005). Ventricular late potentials (VLPs) occurred in 54% of cases. In these patients, TNF-α levels were higher compared to those without VLPs (p<0.05). We may conclude that TNF-α levels might represent and adjunctive criterion for disease staging in patients with myotonic dystrophy type 1, and that elevated TNF levels in DM1 may lead to cardiac fibrosis affecting diastolic function, conduction, and automaticity. © 2002 Elsevier Science B.V. All rights reserved.
2002
cytokines; diastolic function; muscular-dystrophy; myotonic dystrophy type 1; qt-dispersion; tumor necrosis factor-alpha; ventricular late potentials
01 Pubblicazione su rivista::01a Articolo in rivista
Tumor necrosis factor-alpha and myocardial function in patients with myotonic dystrophy type 1 / Mammarella, Antonio; Ferroni, Patrizia; Michele, Paradiso; Francesca, Martini; Paoletti, Vincenzo; Stefania, Morino; Antonini, Giovanni; Gazzaniga, Pierpaolo; Musca, Antonino; Basili, Stefania. - In: JOURNAL OF THE NEUROLOGICAL SCIENCES. - ISSN 0022-510X. - STAMPA. - 201:1-2(2002), pp. 59-64. [10.1016/s0022-510x(02)00193-4]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/251355
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