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

• YAZAKI Masahide

  Third Department of Medicine, Shinshu University School of Medicine, Matsumoto

• TAKEI Yo-ichi

  Third Department of Medicine, Shinshu University School of Medicine, Matsumoto

• KOBAYASHI Keiko

  Department of Molecular Metabolism and Biochemical Genetics, Kagoshima University Graduate School of Medical and Dental Sciences

• SAHEKI Takeyori

  Department of Molecular Metabolism and Biochemical Genetics, Kagoshima University Graduate School of Medical and Dental Sciences

• IKEDA Shu-ichi

  Third Department of Medicine, Shinshu University School of Medicine, Matsumoto

Bibliographic Information

Other Title

• Risk of Worsened Encephalopathy after Intravenous Glycerol Therapy in Patients with Adult-onset Type 2 Citrullinemia(CTLN2)

Abstract

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.<br>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).<br>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.<br>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.

Journal

• Internal Medicine

  Internal Medicine 44 (3), 188-195, 2005

  The Japanese Society of Internal Medicine

Keywords

• type II citrullinemia
• citrin
• glycerol
• mannitol
• brain edema
• cytosolic NADH

Details

• CRID: 1390282679845923584
• NII Article ID: 10015567154
• NII Book ID: AA10827774
• DOI: 10.2169/internalmedicine.44.188
• ISSN: 13497235
• ISSN: 09182918
• NDL BIB ID: 7290626
• PubMed: 15805705
• Web Site: http://id.ndl.go.jp/bib/7290626
• Web Site: https://ndlsearch.ndl.go.jp/books/R000000004-I7290626
• Web Site: http://www.jstage.jst.go.jp/article/internalmedicine/44/3/44_3_188/_pdf
• Web Site: https://search.jamas.or.jp/link/ui/2006014040
• Text Lang: en
• Data Source:
  • JaLC
  • NDL
  • Crossref
  • PubMed
  • CiNii Articles
  • KAKEN
• Abstract License Flag: Disallowed