These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.


Pubmed for Handhelds

PUBMED FOR HANDHELDS

Search MEDLINE/PubMed

  • Title: A case of classical maple syrup urine disease that was successfully managed by living donor liver transplantation.
    Author: Takano C, Ishige M, Ogawa E, Usui H, Kagawa R, Tajima G, Fujiki R, Fukao T, Mizuta K, Fuchigami T, Takahashi S.
    Journal: Pediatr Transplant; 2017 Aug; 21(5):. PubMed ID: 28612395.
    Abstract:
    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.
    [Abstract] [Full Text] [Related] [New Search]