Pegunigalsidase alfa

No UK SPC was retrieved — dose taken from the US prescribing information for ELFABRIO (pegunigalsidase alfa-iwxj); verify against the UK SPC before use. Total infusion volume and the infusion rates for the initial 4-6 infusions are based on actual body weight and differ for ERT-experienced (Table 1) and ERT-naive (Table 2) patients — consult the full prescribing information. If the initial 4-6 infusions are tolerated, the duration of every third infusion may be decreased in decrements of 30 minutes as tolerated. Pretreatment: in ERT-experienced patients who received antihistamines, antipyretics and/or corticosteroids before previous ERT, consider similar pretreatment before the first several infusions, with stepwise reduction/discontinuation after 4-6 tolerated infusions; in ERT-naive patients pretreatment may be considered. Appropriate medical support including cardiopulmonary resuscitation equipment should be readily available during administration. Missed doses: restart as soon as possible, maintaining the 2-week interval thereafter — do not double a dose. Hypersensitivity/infusion-associated reactions: for severe reactions discontinue immediately and treat; for mild to moderate reactions consider holding the infusion for 15-30 minutes or slowing the rate by 25-50%, then resuming at a 25-50% reduced rate as tolerated, increasing by 25% every third infusion until the previous rate is reached. Monitor serum creatinine and urine protein:creatinine ratio (membranoproliferative glomerulonephritis reported).