111,881 research outputs found
Duchenne muscular dystrophy: rational basis, state of the art
No full textAbstract
Duchenne muscular dystrophy (DMD) is an X-linked recessive disease that affects approximately 1 in 3500 male births. Boys with Duchenne have a progressive and predictable muscle deterioration: muscles lack dystrophin, a protein essential for membrane stability, whose absence induces contraction-related membrane damage and activation of the inflammatory cascade leading to muscle failure, necrosis, fibrosis. Although DMD is present at birth, clinical symptoms are not evident until 2-6 years of age. Initial symptoms include leg weakness, increasing spine kyphosis, and a waddle-like gait. Continuous muscle wasting leads to progressively weaker muscles, usually leading DMD patients on wheelchair by the age of 8-12. Scoliosis develops in 90% of boys who use a wheelchair full-time. Progression of muscle degeneration and worsening clinical symptoms lead to death in the late twenties from respiratory/cardiac failure
Gli epigrammi funerari del sepolcreto dei Fadieni (Gambulaga) e i carmi epigrafici del Ferrarese
No full textEsame filologico di una serie di epigrammi funerari rinvenuti in un sepolcreto prediale della zona di Ferrar
Biofilm-dependent airway infections: a role for ambroxol?
No full textBiofilms are a key factor in the development of both acute and chronic airway infections. Their relevance is well established in ventilator associated pneumonia, one of the most severe complications in critically ill patients, and in cystic fibrosis, the most common lethal genetic disease in Caucasians. Accumulating evidence suggests that biofilms could have also a role in chronic obstructive pulmonary disease and their involvement in bronchiectasis has been proposed as well. When they grow in biofilms, microorganisms become multidrug-resistant. Therefore the treatment of biofilm-dependent airway infections is problematic. Indeed, it still largely based on measures aiming to prevent the formation of biofilms or remove them once that they are formed. Here we review recent evidence suggesting that the mucokinetic drug ambroxol has specific anti-biofilm properties. We also discuss how additional pharmacological properties of this drug could be beneficial in biofilm-dependent airway infections. Specifically, we review the evidence showing that: 1-ambroxol exerts anti-inflammatory effects by inhibiting at multiple levels the activity of neutrophils, and 2-it improves mucociliary clearance by interfering with the activity of airway epithelium ion channels and transporters including sodium/bicarbonate and sodium/potassium/chloride cotransporters, cystic fibrosis transmembrane conductance regulator and aquaporins. As a whole, the data that we review here suggest that ambroxol could be helpful in biofilm-dependent airway infections. However, considering the limited clinical evidence available up to date, further clinical studies are required to support the use of ambroxol in these diseases
Duchenne muscle activity evaluation and muscle function preservation: is it possible a prophylactic strategy?
No full textDuchenne muscular dystrophy yields pervasive and progressive muscle mass loss. In the current measures relating to the monitoring of disease progression the following are relevant (i): the type of scale used, (ii) the clinical significance of the attribute being measured and (iii) the mathematical properties of the data provided. The high prevalence of obesity at an early stage of this pathology could result not only from reduced physical activity, but also from low resting energy expenditure, abnormal nutrient utilization or overfeeding. This muscle weakness may be attenuated by regular low-intensity exercise. However, there is a critical lack of data to support appropriate exercise prescription. Because inappropriate activity may exacerbate the dystrophic process, a systematic analysis of muscle function to determine potential exercise load thresholds to avoid injury in dystrophic mice and dogs, and then in humans is recommended
author-bios-SRD-19-0063.R1 – Supplemental material for The Network Structure of Police Misconduct
No full textSupplemental material, author-bios-SRD-19-0063.R1 for The Network Structure of Police Misconduct by George Wood, Daria Roithmayr and Andrew V. Papachristos in Socius</p
Cyclic nucleotide signalling pathways as a potential druggable target for enhancing regeneration potential in dystrophic skeletal muscle
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