总结1例肝糖原累积病合并脓毒症伴冷凝集现象患者的护理体会。肝糖原累积病是罕见的代谢障碍性疾病，发热、感染等因素易诱发急性代谢危象，且患者伴冷凝集现象，其治疗及护理均较为复杂，护理要点包括血糖及营养管理、纠正酸中毒、控制感染、基于循证的目标体温管理、确保输血安全。经过8 d积极治疗和精心护理，患者病情好转并顺利出院，出院后随访6个月，按计划复诊，状态良好。

To summarize the nursing experience of a patient with glycogen storage disease complicated with sepsis and cold agglutination phenomenon. Glycogen storage disease is a rare metabolic disorder，factors such as fever and infection can easily induce acute metabolic crisis，and the patient was accompanied by cold agglutination，its treatment and nursing were complicated. The key points of nursing care included blood sugar and nutrition management，acidosis correction，infection control，evidence-based target body temperature management，ensuring safe blood transfusion. After 8 days of active treatment and careful nursing care，the patient’s condition improved and she was discharged. After 6 months of follow-up，she returned to the hospital as scheduled，the recovery was good.