Treatment With Metformin in Chinese Children With Alport Syndrome

Inclusion Criteria:

• (1) The diagnostic of Alport syndrome-hematuria with or without proteinuria meets oneor more of the following 4 items: i) Immunofluorescence staining of the α5 (IV) chainof the glomerular basement membrane was abnormal; ii) electron microscopy of renalbiopsy showed diffuse changes in the glomerular basement membrane; iii) There was onepathogenic variant in the COL4A5 gene or two pathogenic variants in the COL4A3/COL4A4gene; iv) Family history of Alport syndrome. (2) Age is older than or equal to 10 years old. (3) The 24-hour urine proteinquantification was greater than 150mg and the urine microalbumin-creatinine ratio wasgreater than 30 mg/g. (4) The estimated glomerular filtration rate (24-hour creatinine clearance) wasgreater than 45 mL/min/1.73m2. (5) Accept the maximum tolerated dose of renin-angiotensin-aldosterone system blockers (Blood pressure in the clinic was in the 50th percentile of the same age, same sex andsame height but greater than 90/60mmHg, blood potassium was normal, and the increaseof blood creatinine did not exceed 30%) for at least 3 months.

Exclusion Criteria:

• (1) Vitamin B12 deficiency was uncorrected. (2) Treated with Furosemide, amiloride,and nifedipine. (3) Hypertension, chronic liver disease, chronic heart disease, andchronic kidney disease resulting from causes except Alport syndrome. (4) History of hyperlactatemia. (5) Allergy to metformin. (6) kidney transplant. (7)Poor compliance. (8) Type 1 diabetes.