The number of patients with an indication for RAS-I therapy for Alport syndrome according to the Japanese Society of Pediatric Nephrology and Alport Classification Working Group25 was as follows: 34 (100%) in male XL Alport syndrome; 20 (38.3%) in female XL Alport syndrome; 7 (100%) in AR Alport syndrome; 6 (28.6%) in AD Alport syndrome, and 1 (100%) in digenic Alport syndrome. The gene discussed is MMP19; the disease is Alport syndrome.