To investigate the status of quality of life after liver transplantation in children with genetic metabolic diseases, and to provide a basis for medical staff to develop postoperative health education plans, and also to improve the quality of life of this population.

The convenient sampling method was used to collect data by questionnaire from 65 children diagnosed with genetic metabolic diseases who received liver transplantation in Renji Hospital, Shanghai Jiao Tong University School of Medicine From October 2021 to March 2022. The questionnaire consisted of general information survey and Pediatric Quality of Life Inventoru (PedsQL™3.0) transplantation module. The normally distributed measurement data were compared with group t-test or analysis of variance, while the comparison of non-normally distributed measurement data adopted Kruskal-Wallis or Mann-Whitney test. Using multiple linear regression to analyze the factors affecting the quality of life in children with genetic metabolic diseases after liver transplantation.

The total score of the PedQL™3.0 transplantation module of 65 children with genetic metabolic disorders after liver transplantation was (65±16), with the highest scores in the three dimensions of " medication" , " pain" , and " communication problems" . The scores for " medication" and " communication problems" were 75 (67, 92) and 75 (56, 88), respectively, and the scores for " pain" were (77±16). The score for the dimension of " anxiety towards treatment" is the lowest, at (51±25) points. Girls scored higher in the dimension of " worry" than boys, and the difference was statistically significant (t=-2.079, P<0.05). The scores of the dimensions of " relationship between transplantation and others" and " worry" in children undergoing living donor liver transplantation were higher than those in children undergoing organ donation after the death of citizens liver transplantation, and the differences were statistically significant (t=-2.353 and -1.176, P all <0.05). Children living in first tier cities scored higher in the dimension of "anxiety towards treatment" than those living in non-first tier cities, and the difference was statistically significant (t=-2.190, P<0.05). The results of multiple linear regression analysis showed that living in first tier cities was a factor affecting the quality of life of children with genetic metabolic diseases after liver transplantation (P<0.05).

The quality of life of children with genetic metabolic diseases after liver transplantation needs to be improved, and whether they live in first tier cities is a influencing factor.