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

Mol Genet Metab Rep. 2018 Mar 7:15:71-73. doi: 10.1016/j.ymgmr.2018.02.007. eCollection 2018 Jun.

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: Enzyme replacement therapy; Lysosomal storage disorder; Mucopolysaccharidosis; Totally implantable venous access device.