Case report of living donor liver transplantation for pediatric propionic acidemia combined with dilated cardiomyopathy
10.3760/cma.j.cn101070-20200717-01203
- VernacularTitle:亲属活体肝移植治疗儿童丙酸血症并扩张型心肌病1例
- Author:
Guangpeng ZHOU
1
;
Zhijun ZHU
;
Liying SUN
;
Lin WEI
;
Wei QU
;
Zhigui ZENG
;
Ying LIU
;
Yule TAN
;
Jun WANG
Author Information
1. 首都医科大学附属北京友谊医院肝脏移植中心，国家消化系统疾病临床医学研究中心，北京 100050
- Keywords:
Propionic acidemia;
Cardiomyopathy;
Liver transplantation
- From:
Chinese Journal of Applied Clinical Pediatrics
2021;36(23):1828-1831
- CountryChina
- Language:Chinese
-
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.
