Abstract: Abstract Objective:To assess the health-related quality of life(HRQoL) of children with Alport syndrome. Methods:In this case control study, the cases included children with Alport syndrome who attended the 6th Alport family meeting in China held in 2018, and the controls included children with primary nephrotic syndrome who were admitted to Division of Nephrology, Department of Pediatrics, Peking University First Hospital between October 2017 and February 2018. The demographic, clinical and social data were collected using a self-designed questionnaire. The Chinese version of Pediatric Quality of Life InventoryTM (PedsQLTM) 4. 0 Generic Core Scales were used to assess HRQoL in the participants. Parallel child self-report (5-18 years) and parent proxy-report (2-18 years) forms was included. The trained research assistants guided the questionnaire filling by the same explanatory words and instructions. Results:A total of 91 families(61 families with Alport syndrome and 30 families with primary nephrotic syndrome)were recruited in the study. There were significant differences in the duration of disease, the level of proteinuria, the condition in the last one month, hospitalization frequency, family monthly average income and the impact of disease cost on family between children with Alport syndrome and primary nephrotic syndrome. A total of 81 child self-report forms (54 from Alport syndrome and 27 from primary nephrotic syndrome) and 91 parent proxy-report forms (61 from Alport syndrome and 30 from primary nephrotic syndrome) were collected. There were no significant differences in overall scales and four dimensions between the child-self and parent-proxy reports for both Alport syndrome and primary nephrotic syndrome. Compared with children affected by primary nephrotic syndrome and their parents, no significant differences were observed in overall scales and four subscales of the child-self reports from Alport syndrome, whereas parents of children with Alport syndrome had lower scores in the social subscale. Conclusion:Compared with primary nephrotic syndrome, HRQoL of Alport syndrome was not worse in the assessment by the Chinese version of PedsQLTM 4.0 Generic Core Scales.