Living-related liver transplantation for neonatal-onset propionic acidemia.
case_report · Level V
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- Record sourced from PubMed, PMID 11035841.
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Abstract
We report a child with neonatal-onset propionic acidemia treated with living-related liver transplantation. Despite minimal improvement in the levels of circulating propionyl CoA metabolites, hyperammonemia was corrected, and no episode of metabolic decompensation was experienced after the transplantation was performed. Natural protein intake could be increased from 0.5 g/kg per day to 2 g/kg per day. Anemia was corrected, and the growth rate and mental development improved significantly.
Medical subject headings
- Carboxy-Lyases
- Liver Transplantation
- Metabolic Diseases
- Propionates
- Sodium-Potassium-Exchanging ATPase