关键词: 丙酸血症, 儿童, 肝移植, 重症监护

Abstract: The postoperative nursing experience of two children with propionic acidemia undergoing parent-child liver transplantation was summarized. In view of the problems of multiple organ involvements,metabolic disorders,vascular complications and difficulty of sedation in this group of children,the following interventions were adopted,including monitoring the function of important organs and reporting abnormal conditions timely,the maintain of acid-base balance and being cautious of acidosis and hyperammonemia,strict implementation of anticoagulation therapy to reduce vascular-related complications,effective sedative care to prevent the occurrence of convulsions,accurate administration of immunosuppressants to prevent acute rejection,special formulated diets and control of protein intake. After active treatment and meticulous care,two children were discharged from the hospital after surgery and followed up for more than 1 year with a good prognosis.

Key words: Propionic Acidemia, Child, Liver Transplantation, Intensive Care