Ⅰ型糖原累积病围麻醉期处理1例并文献复习

Peri-anaesthesia management of one case of type I glycogen storage disease and literature review

目的探讨Ⅰ型糖原累积病围麻醉期处理和效果。方法回顾性分析1例Ⅰ型糖原累积病患者行剖腹探查术、左肝肿瘤切除术、右肝肿瘤射频消融术的麻醉处理并结合文献复习，根据Ⅰ型糖原累积病患者病理生理变化进行术前麻醉评估、术中麻醉处理、术后疼痛治疗等进行分析讨论。结果本例患者在全凭静脉全身麻醉下成功的行剖腹探查下肝肿瘤切除术、肝肿瘤射频消融术，术前评估进行医学营养干预：建议生玉米淀粉保持血糖稳定，进行保肝治疗和控制感染；术中根据血气等监测维持内环境和循环稳定；术后进行阿片类药物镇痛；患者生命体征相对平稳，术后随访无相关麻醉并发症，15天后顺利出院。结论Ⅰ型糖原累积病围术期血糖，高乳酸血症的控制和肝功能保护有利于维持患者围术期平稳和临床转归。

Objective To investigate the treatment and effect of type I glycogen storage disease during peri-anaesthesia period. Methods One case of type Ⅰ glycogen storage disease was retrospectively analyzed and underwent laparotomy, left liver resection, right hepatic tumor ablation treatment, anesthesia. The literatures were reviewed. According to the pathophysiological changes of type Ⅰ glycogen storage disease, preoperative anesthesia assessment, intraoperative anesthesia, and postoperative pain treatment were analyzed and discussed. Results The patient was successfully performed laparotomy and liver tumor resection and radiofrequency ablation of liver tumors under total intravenous anesthesia. Preoperative anesthesia assessment indicated that medical nutrition intervention, taking corn starch to keep blood sugar stable, liver protective treatment, and controling infection before the operation, maintaining internal environment and circulation stable blood according to gas monitoring during the operation, and taking analgesic drugs after the operation, should be taken. The patient＇s vital signs were relatively stable. No anesthesia complications were found by postoperative follow-up. The patients was discharged 15 days after hospitalization. Conclusion Perioperative glycemic control, control of lactic acidosis, and protection of liver function in patients with type Ⅰ glycogen storage disease are conducive to maintaining stable perioperative and clinical outcomes.