Risks of long-term port use in enzyme replacement therapy for lysosomal storage disorders

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Abstract

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

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1

Paediatrics and Child Health, University of Pretoria, Steve Biko Academic Unit, Pretoria, South Africa.