Case report of living donor liver transplantation for pediatric propionic acidemia combined with dilated cardiomyopathy / 中华实用儿科临床杂志
Chinese Journal of Applied Clinical Pediatrics
; (24): 1828-1831, 2021.
Article
em Zh
| WPRIM
| ID: wpr-908066
Biblioteca responsável:
WPRO
ABSTRACT
To analyzed a case of pediatric patient with propionic acidemia combined with dilated cardiomyopathy retrospectively, who underwent living donor liver transplantation at the Liver Transplantation Center, Beijing Friendship Hospital, Capital Medical University in March 2019.A 2 years and 6 months female child was admitted to hospital for propionic acidemia.The pretransplant echocardiogram showed left ventricular dilatation and systolic dysfunction, and thus dilated cardiomyopathy was considered.A living donor liver transplant was performed using her mother′s left latera-llobe.On the 14 months postoperatively, the child was on a liberated protein diet, but still required levocarnitine supplementation.Her hepatic and cardiac function returned normal, but growth retardation was still present.During the follow-up period, further propionic acidemia-related complications like metabolic decompensation, or any transplant-related complications were not reported.This case report suggested that liver transplantation is effective on pediatric propionic acidemia combined with cardiomyopathy, which reverses cardiomyopathy, improves cardiac function, relieves strict protein restriction, reduces the risk of metabolic decompensation, and significantly improves quality of life.
Texto completo:
1
Base de dados:
WPRIM
Aspecto:
Patient_preference
Idioma:
Zh
Revista:
Chinese Journal of Applied Clinical Pediatrics
Ano de publicação:
2021
Tipo de documento:
Article