A large number of human diseases are caused by chronic tissue injury with fibrosis potentially leading to organ failure. There is a need for more effective anti-fibrotic therapies. Congenital muscular dystrophy type 1A (MDC1A) is a devastating form of muscular dystrophy caused by laminin α2 chain-deficiency. It is characterized with early inflammation and build-up of fibrotic lesions, both in p...

Muscular dystrophies (MD) constitute a group of inherited disorders, characterized by progressive skeletal muscle weakness and heart involvement. Cardiac disease is common and not necessarily related to the degree of skeletal myopathy; it may be the predominant manifestation with or without any other evidence of muscular disease. Death is usually due to ventricular dysfunction, heart block or m...

Objective. To determine whether there is variability in the attitudes and practices of physicians regarding treatment of respiratory failure in children with spinal muscular atrophy type I (SMA type I) and, if so, whether this variation is associated with professional training. Methods. This was a descriptive, cross-sectional survey mailed to a randomly selected subset of the Child Neurology So...

L-tyrosine, B6 and folic acid are involved in biosynthesis of DOPA and consequently dopamine. The aim of this study was to investigate the antiparkinsonian effect of these agents in perphenazine-induced catatonia in rats. Murprogo method or scored muscular rigidity, which is induced by a phenothiazine, was used to evaluate the antiparkinsonian effect of these agents. A significant decrease in m...

Muscular weakness and muscle wasting may often be observed in critically ill patients on intensive care units (ICUs) and may present as failure to wean from mechanical ventilation. Importantly, mounting data demonstrate that mechanical ventilation itself may induce progressive dysfunction of the main respiratory muscle, i.e. the diaphragm. The respective condition was termed 'ventilator-induced...

YOCARDIAL as well as skeletal muscle lvi involvement occurs in a high proportion of cases of human progressive muscular dystrophy. This cardiac involvement constitutes a specific cardiomyopathy. Clinical evidence of myocardial involvement by the dystrophic process includes labile tachycardia in over 50 per cent of cases, congestive heart failure, various arrhythmias, cardiac murmurs, and cardio...