Background: Difficult clinical encounters pose emotional and behavioural challenges for medical students. Unless resolved, they threaten students' professional competence and well-being. Learning how to humanistically interact with patients perceived as "difficult" is an important component of the developmental process that underlies professional identity formation (PIF).
View Article and Find Full Text PDFPompe disease (PD) is a progressive myopathy caused by the aberrant accumulation of glycogen in skeletal and cardiac muscle resulting from the deficiency of the enzyme acid alpha-glucosidase (GAA). Administration of recombinant human GAA as enzyme replacement therapy (ERT) works well in alleviating the cardiac manifestations of PD but loses sustained benefit in ameliorating the skeletal muscle pathology. The limited efficacy of ERT in skeletal muscle is partially attributable to its inability to curb the accumulation of new glycogen produced by the muscle enzyme glycogen synthase 1 (GYS1).
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