Risk of Worsened Encephalopathy after Intravenous Glycerol Therapy in Patients with Adult-onset Type II Citrullinemia (CTLN2)

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概要

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• 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 1 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.

• 社団法人　日本内科学会の論文
• 2005-03-01

著者

• IKEDA Shu-ichi

  Third Department of Medicine, Shinshu University School of Medicine

• KOBAYASHI Keiko

  Department of Geriatric and Environmental Dermatology, Nagoya City University Graduate School of Med

• SAHEKI Takeyori

  Department of Biochemistry, Faculty of Medicine, Kagoshima University

• YAZAKI Masahide

  Third Department of Medicine, Shinshu University School of Medicine

• TAKEI Yo-ichi

  Third Department of Medicine, Shinshu University School of Medicine

• Takei Yo-ichi

  Third Department Of Medicine Shinshu University School Of Medicine

• Ikeda Shu-ichi

  Third Department Of Medicine Shinshu University School Of Medicine

• Kobayashi Keiko

  Department Of Biochemical Pharmacology Faculty Of Pharmaceutical Sciences Chiba University

• Saheki Takeyori

  Department Of Biochemistry Faculty Of Medicine Kagoshima University

• Saheki Takeyori

  Department Of Molecular Metabolism And Biochemical Genetics Kagoshima University Graduate School Of

• Yazaki Masahide

  Third Department Of Medicine Shinshu University School Of Medicine

• Kobayashi Keiko

  Department Of Molecular Metabolism And Biochemical Genetics Kagoshima University Graduate School Of

• Kobayashi Keiko

  Dep. Of Molecular Metabolism And Biochemical Genetics Kagoshima Univ. Graduate School Of Medical And

• Saheki Takeyori

  Inst. For Health Sciences Tokushima Bunri Univ.

• Ikeda Shu-ihci

  Dep. Of Medicine (neurology And Rheumatology) Shinshu Univ. School Of Medicine

• Ikeda Shu-ichi

  Third Department Of Medicine (neurology And Rheumatology) Shinshu University School Of Medicine

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