The Efficacy and Safety of Dapagliflozin in the Treatment of Hereditary Kidney Disease With Proteinuria in Children

Inclusion Criteria:

• Confirmed diagnosis of hereditary kidney disease (identification of pathogenic genesthrough molecular genetic testing; for Alport syndrome, molecular diagnosis is notnecessarily required if diagnosed based on clinical and pathological findings; forthose with a clear family history and a high clinical suspicion of hereditary kidneydisease).

• 24 - hour urinary protein level > 0.2 g or urinary protein to creatinine ratio (UPCR) > 0.2 mg/mg.

• Calculate the estimated glomerular filtration rate (eGFR) using the Schwartz formula (36.5 * height in cm / serum creatinine in μmol/L), with eGFR ≥ 60 ml/min/1.73 m².

• Stable use of the basic treatment drug RAASi (including ACEI/ARB) for more than 4weeks, and no dosage adjustment during the treatment period.

• Willingness to sign the informed consent form.

Exclusion Criteria:Exclusion applies if any of the following criteria are met:

• Treatment with hormones/immunosuppressive agents within the previous 4 weeks.

• Treatment with SGLT2 inhibitors within the previous 4 weeks.

• Comorbid diabetes.

• Uncontrolled urinary tract infection.

• Evidence of urinary tract obstruction such as dysuria.

• Blood pressure below the 5th percentile for the same gender, age, and height.

• Organ transplantation.

• Tumor.

• Presence of any of the following definite evidence of liver disease: ALT/ASTreaching 2 times the normal value, hepatic encephalopathy, esophageal varices, orportal shunt surgery.

• Comorbid medical conditions that may affect drug absorption, distribution,metabolism, and excretion, including but not limited to any of the following: activeinflammatory bowel disease within the past 6 months, history of majorgastrointestinal surgery (such as gastrectomy, gastroenterostomy, intestinalresection), gastrointestinal ulcer, gastrointestinal or rectal bleeding within thepast 6 months, pancreatic injury or pancreatitis within the past 6 months.

• Subjects at risk of dehydration or volume depletion, which may affect drug efficacyor safety.

• Participation in other drug trials within the previous 4 weeks.

• Blood loss exceeding 400 ml within the previous 8 weeks.

• Poor past medication compliance or unwillingness to complete the trial.

• Any other medical conditions that may place the patient at a higher risk due toparticipation in this study.