*Purpose: Liver transplant (LT) can increase quality and quantity of life for patients with inherited metabolic diseases (IMDs) who suffer severe and unpredictable complications despite strict dietary and medical management. Due to advancements in surgical techniques and immunosuppression, LT has been increasingly used as a therapeutic option for IMDs to compensate for enzyme insufficiencies. However, the risks of LT must also be considered for each individual patient. We aimed in this study at gaining a better understanding of the potential benefits and complications of LT in patients with IMDs

*Methods: We retrospectively analyzed metabolic outcomes, hospitalizations, dietary treatment, and mortality in 57 individuals with IMDs from the Children’s National Health System who received LTs at the MedStar Georgetown University Hospital between 2002 and 2017.

*Results: In the patients with intoxication-type IMDs (i.e.maple syrup urine disease, proprionic acidemia, methylmalonic aciduria and urea cycle disorders), there was a relaxation in diet, decreased hospitalizations for metabolic decompensations, and discontinuation of nitrogen scavengers (if used prior to transplant). Survival rate was 100% in those with intoxication-type IMDs who underwent LT in the last 5 years.

*Conclusions: For individuals with moderate to severe presentations of intoxication-type IMDs, LT is a viable treatment option.

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