A case of classical maple syrup urine disease that was successfully managed by living donor liver transplantation

Chika Takano, Mika Ishige, Erika Ogawa, Hiromi Usui, Reiko Kagawa, Go Tajima, Ryoji Fujiki, Toshiyuki Fukao, Koichi Mizuta, Tatsuo Fuchigami, Shori Takahashi

研究成果: ジャーナルへの寄稿記事査読

7 被引用数 (Scopus)

抄録

Classical MSUD is often fatal without appropriate medical interventions because of metabolic crisis. There are numerous reports suggesting the therapeutic potential of deceased donor liver transplantation for MSUD. However, the usefulness of LDLT for MSUD is unknown. We report a case of classical MSUD, which was successfully managed by LDLT from the patient's father at 1 year of age. Abnormal brain findings, which were cured with effective treatment, gradually disappeared after LDLT. The patient then developed normally. Findings from this case suggest the importance of LDLT for maintaining low leucine levels and subsequent normal neurological development. Although LDLT involves a modest surgical insult, LDLT with a related donor achieves acceptable leucine levels for life.

本文言語英語
論文番号e12948
ジャーナルPediatric Transplantation
21
5
DOI
出版ステータス出版済み - 8月 2017

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