Overall, more than one-half of the children (57%, 12/21 patients) died during the observation period (patients No 1–6, 8, 9, 14, 18, 19, and one child without any pathogenic variants in SMARCAL1); three of them had not reached chronic kidney disease (CKD) stage 5 (patients No 1, 4, and 6). The gene discussed is SMARCAL1; the disease is chronic kidney disease.