Living-related liver transplantation for neonatal-onset propionic acidemia.

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.

• Authors: T Yorifuji, J Muroi, A Uematsu, T Nakahata, H Egawa, K Tanaka
• Journal: The Journal of pediatrics (J Pediatr) Vol. 137 Issue 4 Pg. 572-4 (Oct 2000) ISSN: 0022-3476 [Print] United States
• PMID: 11035841 (Publication Type: Case Reports, Journal Article)

Chemical References

• Propionates
• Carboxy-Lyases
• Sodium-Potassium-Exchanging ATPase
• Methylmalonyl-CoA Decarboxylase
• propionic acid

Topics

• Carboxy-Lyases (deficiency)
• Female
• Humans
• Infant
• Liver Transplantation
• Metabolic Diseases (surgery)
• Methylmalonyl-CoA Decarboxylase
• Propionates (blood)
• Sodium-Potassium-Exchanging ATPase