Treatment Beliefs Reflect Unmet Clinical Needs in Lysosomal Storage Diseases: An Opportunity for a Patient-Centered Approach.

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Tác giả: Marion M M G Brands, Eleonore M Corazolla, Carla E M Hollak, Mirjam Langeveld, Barbara Sjouke

Ngôn ngữ: eng

Ký hiệu phân loại: 623.8728 Nautical engineering and seamanship

Thông tin xuất bản: United States : JIMD reports , 2025

Mô tả vật lý:

Bộ sưu tập: NCBI

ID: 682467

Despite life-long pharmacotherapy for many people affected by lysosomal storage diseases, no data are available on their beliefs about their treatments. Therapeutic options range from disease-specific, with varying levels of effectiveness, to purely supportive. This spectrum is illustrated by the three diseases Gaucher disease type 1 (effective disease-specific therapies), Fabry disease (disease-specific therapies with variable effectiveness), and mucopolysaccharidosis type III A/B (supportive care only). Employing the Necessity-Concerns Framework of the Beliefs in Medicine Questionnaire, we investigated intra- and intergroup variability in adults with Gaucher disease type 1, Fabry disease, and parents of children with mucopolysaccharidosis type III A/B. Participants rated
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