Yorifuji T, Muroi J, Uematsu A, Nakahata T, Egawa H, Tanaka K
Department of Pediatrics, Kyoto University Hospital, Shogoin Sakyo, Kyoto, Japan.
J Pediatr. 2000 Oct;137(4):572-4. doi: 10.1067/mpd.2000.108391.
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.
我们报告了一名患有新生儿期丙酸血症并接受活体亲属肝移植治疗的儿童。尽管循环中丙酰辅酶A代谢物水平改善甚微，但高氨血症得到纠正，移植后未发生代谢失代偿事件。天然蛋白质摄入量可从每天0.5克/千克增加到每天2克/千克。贫血得到纠正，生长速度和智力发育显著改善。
