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2017 ; 34
(ä): 63-70
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Delayed Infusion Reactions to Enzyme Replacement Therapies
#MMPMID27557812
Karimian Z
; Whitley CB
; Rudser KD
; Utz JRJ
JIMD Rep
2017[]; 34
(ä): 63-70
PMID27557812
show ga
BACKGROUND: There are currently ten intravenous enzyme replacement therapy (ERT)
products available for the treatment of eight different lysosomal diseases (LD)
in the USA. Additional ERT products are in clinical trials. The most common ERT
adverse events are infusion reactions (IR). While IR are often defined as
hypersensitivity or anaphylactoid reactions occurring concurrently with (i.e.,
during) infusion administration (CIR), there exists the potential for delayed
infusion reactions (DIR), which present after completion of infusion
administration. HYPOTHESIS: Concurrent infusion reactions (CIR) are not the only
infusion reactions associated with enzyme therapy. METHODS: This study evaluated
the occurrence of infusion reactions in 46 patients with LD who had received ERT
for a minimum of 2 years. Infusion reactions were evaluated according to
symptoms, time of onset, and duration of reactions. The frequency of infusion
reactions with each ERT product was compared to that reported in the FDA-approved
product package insert. RESULTS AND CONCLUSIONS: In this study, DIR were observed
and occurred as often as CIR in the study population, despite not being
characterized or reported in most ERT product package inserts. Effective methods
for managing DIR and CIR differed, thus emphasizing the importance of monitoring
for both types of infusion reactions in order to optimize outcomes for patients
using ERT.