目的 总结法布雷病患者酶替代治疗过程中输液相关反应的观察及护理经验。 方法 回顾性分析2020年9月至2024年3月，山东省某三级甲等医院收治的法布雷病患者21例，在酶替代治疗期间发生输液相关反应的临床表现、护理措施及转归情况。 结果 共21例患者接受酶替代治疗327次，其中13例患者输注注射用阿加糖酶β共112次，9例患者输注阿加糖酶α注射用浓溶液共215次，且有1例患者先后分别接受了注射用阿加糖酶β和阿加糖酶α注射用浓溶液的治疗。期间，4例患者共发生5次输液相关反应，表现为发热、乏力、肢端疼痛加重、肢端麻木及触觉下降，经对症处理后均能继续治疗，未出现严重输液相关反应。 结论 法布雷病患者在酶替代治疗中，严重输液相关反应较为罕见，通常经对症处理后可继续治疗。

Objective To summarize the infusion-related reactions and nursing of enzyme replacement therapy in patients with Fabry disease.Methods The clinical manifestations,nursing and outcomes of infusion-related reactions were retrospectively analyzed in 21 patients with Fabry disease undergoing enzyme replacement therapy from September 2020 to March 2024 admitted to our center.Results A total of 21 patients underwent 327 infusions,including 112 infusions of agalsidase beta for 13 patients and 215 infusions of agalsidase alpha for 9 patients,of which 1 patient underwent infusions of both drugs respectively.A total of 5 infusion-related reactions occurred in 4 patients,including fever,fatigue,aggravated limb pain,numbness and decreased tactile sensation in extremities.All the patients continued infusions after symptomatic treatment,and no severe infusion-related reactions occurred.Conclusions Severe infusion-related reactions of enzyme replacement therapy in patients with Fabry disease rarely occur and can be usually continued after symptomatic treatment.

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