Search results for "Myopathy"
showing 10 items of 352 documents
A recommended practical approach to the management of anthracycline-based chemotherapy cardiotoxicity: an opinion paper of the working group on drug …
2016
Anthracyclines are the mainstay of treatment of a variety of haematological malignancies and solid tumours. Unfortunately, the clinical use of these drugs is limited by cumulative, dose-related cardiotoxicity which may ultimately lead to a severe and irreversible form of cardiomyopathy. Thus, there is an increasing need for close cooperation among cardiologists, oncologists and haemato-oncologists. As anthracyclines save lives, the logical goal of this cooperation, besides preventing or mitigating cardiotoxicity, is to promote an acceptable balance between the potential cardiac side effects and the vital benefit of anticancer treatment. This manuscript, which is specifically addressed to th…
Outcome of patients with classical infantile pompe disease receiving enzyme replacement therapy in Germany
2015
Enzyme replacement therapy (ERT) has been shown to improve outcome in classical infantile Pompe disease. The purpose of this study was to assess mortality, morbidity, and shortcomings of ERT in a larger cohort of patients treated outside clinical trials. To accomplish this, we retrospectively analyzed the data of all 23 subjects with classical infantile Pompe disease having started ERT in Germany between January 2003 and December 2010.Ten patients (43%) deceased and four others (17%) became ventilator dependent. Seven infants (30.5%) made no motor progress at all, while seven (30.5%) achieved free sitting, and nine (39%) gained free walking. Besides all the seven patients (100%) attaining n…
Prognosis of adults with left ventricular non compaction: Results from a prospective multricentric french study
2021
Background Left ventricular non compaction (LVNC) is a rare cause of cardiomyopathy related to abnormal in utero myocardial development, leading to prominent trabeculations. Its prognosis remains unclear with conflicting data and mortality ranging from 2 to 38%. Purpose The aim of this study was to define the prognosis of newly diagnosed LVNC as compared to recently diagnosed dilated cardiomyopathy (DCM). Methods A prospective multicentric study with a 2-year follow-up was designed. The primary endpoint combined cardiovascular death, heart transplantation and hospitalisation for cardiovascular events. Three groups of patients were defined: Group A: new cases of LVNC, Group B: new cases of D…
Impact of pericardial effusion on cardiac mechanics in patients with dilated cardiomyopathy
2013
Dilated cardiomyopathy (CDM) is a degenerative disease of the myocardium accompanied by left ventricular (LV) remodeling, resulting in an impaired pump performance. Differently, pericardial effusion(PE) is a liquid accumulation in the pericardial cavity, which may inhibit blood filling of heart chambers. Clinical evidence show that PE may improve pump performance in patients with CDM. Therefore, this study aims to assess wall stress and global function of patients with CDM, PE as compared to healthy patient. These findings suggests that CDM has an important implication in the mechanical changes of LV and right ventricle by increasing wall stress and reducing pump function. Conversely, PE de…
Expansion of specific alphabeta+ T-cell subsets in the myocardium of patients with myocarditis and idiopathic dilated cardiomyopathy associated with …
2003
Idiopathic dilated cardiomyopathy (IDC) is one of the major causes of death in humans and has been linked to Coxsackievirus B (CVB) infection. The aim of this study was to analyze phenotypes of heart-infiltrating immune cells in patients suffering from myocarditis and IDC associated with CVB infections. We found that the myocardium of these patients was infiltrated by CD4(+) and CD8(+) T lymphocytes as well as macrophages. Evidence of CVB3/4 infections was also found. In the majority of patients, the T-cell receptor repertoire (TCR) of the infiltrating lymphocytes was restricted, with a polyclonal expansion of the Vbeta7 gene family. We also found that human leukocyte antigen (HLA) class II…
Circulating Irisin Levels as a Marker of Osteosarcopenic-Obesity in Cushing’s Disease
2020
Purpose: To evaluate circulating irisin levels in patients with active and controlled Cushing’s disease (CD). Design: Forty-four patients with CD evaluated during the active phase and after 12 months of biochemical remission and 40 controls were recruited. Methods: Phenotypic, anthropometric, hormonal and metabolic parameters, including insulin sensitivity estimation by homeostatic model of insulin resistance, Matsuda index and oral disposition index and circulating irisin levels were evaluated. Results: Patients with active CD showed lower irisin levels compared to controls (p<0.001) and controlled CD (p<0.001). The independent variables significantly associated with irisin were wais…
Qualitative and Quantitative Analysis of Cardiac Progenitor Cells in Cases of Myocarditis and Cardiomyopathy
2018
We aimed to identify and quantify CD117+ and CD90+ endogenous cardiac progenitor cells (CPC) in human healthy and diseased hearts. We hypothesize that these cells perform a locally acting, contributing function in overcoming medical conditions of the heart by endogenous means. Human myocardium biopsies were obtained from 23 patients with the following diagnoses: Dilatative cardiomyopathy (DCM), ischemic cardiomyopathy (ICM), myocarditis, and controls from healthy cardiac patients. High-resolution scanning microscopy of the whole slide enabled a computer-based immunohistochemical quantification of CD117 and CD90. Those signals were evaluated by Definiens Tissue Phenomics® Technology. Co-loca…
Happy heart syndrome. frequency, characteristics, and outcome of takotsubo syndrome triggered by positive life events
2021
Background: The association with a preceding stressor is a characteristic feature of takotsubo syndrome (TTS). Negative emotions before TTS are common and led to the popular term "broken heart syndrome." In contrast, pleasant triggers ("happy heart syndrome") are rare and are scarcely investigated. Objectives: The authors analyzed the frequency, clinical characteristics, and prognostic implications of positive emotional stressors in the multicenter GEIST (GErman-Italian-Spanish Takotsubo) Registry. Methods: Patients enrolled in the registry were categorized according to their stressors. This analysis compared patients with pleasant emotional events with patients with negative emotional even…
Up-and-Coming Markers: Myeloperoxidase, a Novel Biomarker Test for Heart Failure and Acute Coronary Syndrome Application?
2008
Myeloperoxidase (MPO) is a mammalian enzyme responsible for generation of hypochlorite. The advantage of myeloperoxidase for use as a biomarker in the setting of heart failure and acute coronary syndrome is the early increase of MPO concentration in response to the acute event. In the setting of heart failure the reported independency of coronary artery disease and general inflammation, as indicated by MPO concentration in comparison to other inflammatory markers or in subgroups of patients with ischemic and non-ischemic cardiomyopathy, has to be highlighted. In terms of ACS, inclusion of MPO into a multiple marker strategy might add to enhance diagnosis and therapy decision making. Therefo…
Could thiazolidinediones increase the risk of heart failure in Friedreich's ataxia patients?
2011
Clinical evidence and the recent decisions of the European Medicines Agency and the Food and Drug Administration challenge the safety of thiazolidinediones treatment. Recently, this treatment has been suggested for Friedreich's ataxia because thiazolidinediones improve neurological symptoms. Hypertrophic cardiomyopathy is the most prevalent cardiac feature and the cause of premature death in Friedreich's ataxia patients. We recommend that therapy with peroxisome proliferator-activated receptor-gamma agonists like thiazolidinediones be taken with caution, as they cause a decrease in the number of fast fibers and an increase in mitochondrial biogenesis in cardiac muscle because of the inducti…