Risks of long-term port use in enzyme replacement therapy for lysosomal storage disorders
Totally implantable vascular access devices (TIVADs) are commonly used in conjunction with enzyme replacement therapy (ERT) for lysosomal storage disorders (LSDs). This case series describes potential complications associated with long-term TIVAD use, such as compromise of skin integrity, infection,...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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Elsevier
2018-06-01
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| Series: | Molecular Genetics and Metabolism Reports |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2214426918300156 |
| _version_ | 1811256238591180800 |
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| author | Christian J. Hendriksz Paul Harmatz Roberto Giugliani Jane Roberts G. Suren Arul |
| author_facet | Christian J. Hendriksz Paul Harmatz Roberto Giugliani Jane Roberts G. Suren Arul |
| author_sort | Christian J. Hendriksz |
| collection | DOAJ |
| description | Totally implantable vascular access devices (TIVADs) are commonly used in conjunction with enzyme replacement therapy (ERT) for lysosomal storage disorders (LSDs). This case series describes potential complications associated with long-term TIVAD use, such as compromise of skin integrity, infection, or port failures. Best practices and skilled specialists are essential for minimizing complications from long-term TIVAD use for ERT. Keywords: Mucopolysaccharidosis, Enzyme replacement therapy, Totally implantable venous access device, Lysosomal storage disorder |
| first_indexed | 2024-04-12T17:37:07Z |
| format | Article |
| id | doaj.art-ddbba806f0f346248ca095390380ac63 |
| institution | Directory Open Access Journal |
| issn | 2214-4269 |
| language | English |
| last_indexed | 2024-04-12T17:37:07Z |
| publishDate | 2018-06-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Molecular Genetics and Metabolism Reports |
| spelling | doaj.art-ddbba806f0f346248ca095390380ac632022-12-22T03:22:57ZengElsevierMolecular Genetics and Metabolism Reports2214-42692018-06-01157173Risks of long-term port use in enzyme replacement therapy for lysosomal storage disordersChristian J. Hendriksz0Paul Harmatz1Roberto Giugliani2Jane Roberts3G. Suren Arul4Salford Royal Foundation NHS Trust, Manchester, UK; University of Pretoria, Department of Paediatrics and Child Health, Steve Biko Academic Unit, Pretoria, South Africa; Corresponding author at: The Mark Holland Metabolic Unit, Salford Royal Foundation NHS Trust, Ladywell NW2 - 2nd Floor Room 112, Salford, Manchester M6 8HD, UK.UCSF Benioff Children's Hospital Oakland, Oakland, CA, USAMedical Genetics Service, HCPA, Department of Genetics, UFRGS and INAGEMP, Porto Alegre, BrazilSt. Mary's Hospital, Manchester, UKBirmingham Children's Hospital, Birmingham, UKTotally implantable vascular access devices (TIVADs) are commonly used in conjunction with enzyme replacement therapy (ERT) for lysosomal storage disorders (LSDs). This case series describes potential complications associated with long-term TIVAD use, such as compromise of skin integrity, infection, or port failures. Best practices and skilled specialists are essential for minimizing complications from long-term TIVAD use for ERT. Keywords: Mucopolysaccharidosis, Enzyme replacement therapy, Totally implantable venous access device, Lysosomal storage disorderhttp://www.sciencedirect.com/science/article/pii/S2214426918300156 |
| spellingShingle | Christian J. Hendriksz Paul Harmatz Roberto Giugliani Jane Roberts G. Suren Arul Risks of long-term port use in enzyme replacement therapy for lysosomal storage disorders Molecular Genetics and Metabolism Reports |
| title | Risks of long-term port use in enzyme replacement therapy for lysosomal storage disorders |
| title_full | Risks of long-term port use in enzyme replacement therapy for lysosomal storage disorders |
| title_fullStr | Risks of long-term port use in enzyme replacement therapy for lysosomal storage disorders |
| title_full_unstemmed | Risks of long-term port use in enzyme replacement therapy for lysosomal storage disorders |
| title_short | Risks of long-term port use in enzyme replacement therapy for lysosomal storage disorders |
| title_sort | risks of long term port use in enzyme replacement therapy for lysosomal storage disorders |
| url | http://www.sciencedirect.com/science/article/pii/S2214426918300156 |
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