Successful living donor liver transplantation for classical maple syrup urine disease

Toshihiro Yasui, Tatsuya Suzuki, Fujio Hara, Shunsuke Watanabe, Naoko Uga, Atsuki Naoe, Tetsushi Yoshikawa, Tetsuya Ito, Yoko Nakajima, Hiroki Miura, Atsushi Sugioka, Yutaro Kato, Takamasa Tokoro, Yoshinao Tanahashi, Mureo Kasahara, Akinari Fukuda, Hiroki Kurahashi

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

MSUD is an autosomal recessive condition characterized by a deficiency in the enzyme, BCKDH, which catalyzes the breakdown of BCAAs. If left untreated, MSUD can result in mental retardation, central nervous system disorders, and even death. Most patients with MSUD are treated with a restricted protein diet and milk from which BCAAs have been removed. LT has been shown effective in patients with MSUD. This report describes the case of a 15-month-old boy who received a liver graft from his mother. Transplantation was successful, and the patient was then able to ingest a normal diet. Despite episodes of acute rejection, chylous ascites, and high fever (40 °C), he has shown no evidence of MSUD recurrence. These findings indicate that patients with MSUD can be successfully treated by LDLT, even when the donor is a heterozygous carrier of a mutated BCKDH gene.

Original languageEnglish
Pages (from-to)707-710
Number of pages4
JournalPediatric Transplantation
Volume20
Issue number5
DOIs
Publication statusPublished - 01-08-2016

Fingerprint

Maple Syrup Urine Disease
Living Donors
Liver Transplantation
Chylous Ascites
Protein-Restricted Diet
Central Nervous System Diseases
Intellectual Disability
Milk
Fever
Transplantation
Mothers
Tissue Donors
Diet
Transplants
Recurrence
Liver
Enzymes
Genes

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Transplantation

Cite this

Yasui, Toshihiro ; Suzuki, Tatsuya ; Hara, Fujio ; Watanabe, Shunsuke ; Uga, Naoko ; Naoe, Atsuki ; Yoshikawa, Tetsushi ; Ito, Tetsuya ; Nakajima, Yoko ; Miura, Hiroki ; Sugioka, Atsushi ; Kato, Yutaro ; Tokoro, Takamasa ; Tanahashi, Yoshinao ; Kasahara, Mureo ; Fukuda, Akinari ; Kurahashi, Hiroki. / Successful living donor liver transplantation for classical maple syrup urine disease. In: Pediatric Transplantation. 2016 ; Vol. 20, No. 5. pp. 707-710.
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Yasui, T, Suzuki, T, Hara, F, Watanabe, S, Uga, N, Naoe, A, Yoshikawa, T, Ito, T, Nakajima, Y, Miura, H, Sugioka, A, Kato, Y, Tokoro, T, Tanahashi, Y, Kasahara, M, Fukuda, A & Kurahashi, H 2016, 'Successful living donor liver transplantation for classical maple syrup urine disease', Pediatric Transplantation, vol. 20, no. 5, pp. 707-710. https://doi.org/10.1111/petr.12738

Successful living donor liver transplantation for classical maple syrup urine disease. / Yasui, Toshihiro; Suzuki, Tatsuya; Hara, Fujio; Watanabe, Shunsuke; Uga, Naoko; Naoe, Atsuki; Yoshikawa, Tetsushi; Ito, Tetsuya; Nakajima, Yoko; Miura, Hiroki; Sugioka, Atsushi; Kato, Yutaro; Tokoro, Takamasa; Tanahashi, Yoshinao; Kasahara, Mureo; Fukuda, Akinari; Kurahashi, Hiroki.

In: Pediatric Transplantation, Vol. 20, No. 5, 01.08.2016, p. 707-710.

Research output: Contribution to journalArticle

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AU - Yasui, Toshihiro

AU - Suzuki, Tatsuya

AU - Hara, Fujio

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AU - Naoe, Atsuki

AU - Yoshikawa, Tetsushi

AU - Ito, Tetsuya

AU - Nakajima, Yoko

AU - Miura, Hiroki

AU - Sugioka, Atsushi

AU - Kato, Yutaro

AU - Tokoro, Takamasa

AU - Tanahashi, Yoshinao

AU - Kasahara, Mureo

AU - Fukuda, Akinari

AU - Kurahashi, Hiroki

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