Replacing the enzyme alpha-L-iduronidase at birth ameliorates symptoms in the brain and periphery of dogs with mucopolysaccharidosis type I.

Mucopolysaccharidosis type I (MPS I) is a lysosomal storage disease caused by loss of activity of α-l-iduronidase and attendant accumulation of the glycosaminoglycans dermatan sulfate and heparan sulfate. Current treatments are suboptimal and do not address residual disease including corneal clouding, skeletal deformities, valvular heart disease, and cognitive impairment. We treated neonatal dogs with MPS I with intravenous recombinant α-l-iduronidase replacement therapy at the conventional 0.

Evaluating consumer satisfaction in residential continuing care settings.

The outcome of the project reported on here is a client-centered consumer satisfaction questionnaire designed to evaluate new models of residential continuing care in Alberta, Canada. Satisfaction is defined as a multi-dimensional construct that is grounded in the consumer's experience. Consultation with the clients of the services during development of the instrument ensured that characteristics important to the clients were assessed.