The effects of different test methods on the just noticeable difference of clarity index for music.

The just noticeable differences (JNDs) of room acoustics metrics are necessary for research and design of performing arts venues. The goal of this work was to evaluate the effects of different testing methods on the measured JND of clarity index for music (C80). An initial study was conducted to verify the findings of other published works that the C80 JND is approximately 1 dB, as currently listed in ISO 3382:2009 (International Organization for Standardization, Switzerland, 2009), however, the results suggested a higher value.

Management of maxillofacial injuries with severe oronasal hemorrhage: a multicenter perspective.

Background: Airway establishment and hemorrhage control may be difficult to achieve in patients with massive oronasal bleeding from maxillofacial injuries. This study was formulated to develop effective algorithms for managing these challenging injuries.

Methods: Trauma registries from nine trauma centers were queried over a 7-year period for injuries with abbreviated injury scale face >/= 3 and transfusion of >/=3 units of blood within 24 hours.

Autophagy and mistargeting of therapeutic enzyme in skeletal muscle in Pompe disease.

Enzyme replacement therapy (ERT) became a reality for patients with Pompe disease, a fatal cardiomyopathy and skeletal muscle myopathy caused by a deficiency of glycogen-degrading lysosomal enzyme acid alpha-glucosidase (GAA). The therapy, which relies on receptor-mediated endocytosis of recombinant human GAA (rhGAA), appears to be effective in cardiac muscle, but less so in skeletal muscle. We have previously shown a profound disturbance of the lysosomal degradative pathway (autophagy) in therapy-resistant muscle of GAA knockout mice (KO).

Autophagy and lysosomes in Pompe disease.

In Pompe disease, a deficiency of lysosomal acid alpha-glucosidase, intralysosomal glycogen accumulates in multiple tissues, with skeletal and cardiac muscle most severely affected.(1) Complete enzyme deficiency results in rapidly progressive infantile cardiomyopathy and skeletal muscle myopathy that is fatal within the first two years of life. Patients with partial enzyme deficiency suffer from skeletal muscle myopathy and experience shortened lifespan due to respiratory failure.