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

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

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

Author(s) -

Takano Chika,

Ishige Mika,

Ogawa Erika,

Usui Hiromi,

Kagawa Reiko,

Tajima Go,

Fujiki Ryoji,

Fukao Toshiyuki,

Mizuta Koichi,

Fuchigami Tatsuo,

Takahashi Shori

Publication year - 2017

Publication title -

pediatric transplantation

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 0.457

H-Index - 69

eISSN - 1399-3046

pISSN - 1397-3142

DOI - 10.1111/petr.12948

Subject(s) - maple syrup urine disease , medicine , liver transplantation , transplantation , living donor liver transplantation , surgery , leucine , intensive care medicine , pediatrics , amino acid , biochemistry , chemistry

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.

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