AI Article Synopsis
- Classical MSUD can be life-threatening without proper medical treatment, and while deceased donor liver transplants have shown promise, the effectiveness of living donor liver transplants (LDLT) for this condition was previously unclear.
- A case study of a 1-year-old patient with classical MSUD demonstrated successful management through an LDLT from the father, leading to the resolution of abnormal brain findings and normal development.
- The results indicate that LDLT is crucial for keeping leucine levels low, which is essential for typical neurological growth, and although it involves some surgical risks, it can provide a long-term solution for managing MSUD.
Article 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.
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| http://dx.doi.org/10.1111/petr.12948 | DOI Listing |
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