Risk of worsened encephalopathy after intravenous glycerol therapy in patients with adult-onset type II citrullinemia (CTLN2)
Risk of worsened encephalopathy after intravenous glycerol therapy in patients with adult-onset type II citrullinemia (CTLN2)
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DOI：
10.2169/internalmedicine.44.188
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发表时间：
2005-03-01
影响因子：
1.2
通讯作者：
Ikeda, S
中科院分区：
文献类型：
--
作者：
Yazaki, M;Takei, Y;Ikeda, S
目的报道用甘油渗剂治疗柠檬酸缺乏所致的成人II型瓜氨酸血症(CTLN2)患者的脑病恶化风险。患者与方法对3例CTLN2患者进行了回顾性研究，其中1例男性患者和1例女性患者分别接受了10%甘油和20%D-甘露醇治疗脑病相关性脑水肿，40岁男性患者仅接受20%D-甘露醇治疗。此外，我们还对已报道的11例CTLN2患者(8例用10%甘油治疗，2例用10%甘油加20%甘露醇治疗，1例用20%甘露醇治疗)进行了回顾性研究。结果12例用10%甘油治疗的患者，其中2例死于快速加重的脑病和脑水肿。另一方面，2例仅接受20%D-甘露醇治疗的患者，包括我们的1例患者，恢复了脑水肿的消失。结论在CTLN2患者中，甘油输注似乎与脑病本身的加重有关，应该只使用甘露醇来治疗这种疾病患者的脑水肿。
Objective We report a risk of worsening of encephalopathy by glycerol infusion when this osmotic agent is used for the treatment of brain edema in patients with adult-onset type II citrullinemia (CTLN2) caused by citrin deficiency.Patients and Methods We performed a retrospective investigation of 3 patients with CTLN2 treated for brain edema at our institute: a 31-year-old male patient and a 40-year-old female patient received treatment for encephalopathy-related brain edema with 10% glycerol infusion and 20% D-mannitol, and a 40-year-old male patient received only 20% D-mannitol infusion. In addition, we also performed a retrospective study in 11 CTLN2 patients reported previously (8 patients treated with 10% glycerol, 2 treated with 10% glycerol and 20% mannitol, and I treated with 20% mannitol).Results The 12 patients treated with 10% glycerol, including 2 of our patients, died due to rapidly deteriorating encephalopathy and brain edema. On the other hand, the 2 patients who received only 20% D-mannitol, including one of our patients, recovered with the disappearance of brain edema.Conclusion In CTLN2 patients, glycerol infusion seems to be associated with exacerbation of encephalopathy itself and only mannitol should be used for the treatment of brain edema in patients with this disorder.