Safety and Efficacy of ACEI in Alport Syndrome Patients With COL4A3/COL4A4/COL4A5 Variants

Safety and Efficacy of Early Angiotensin-converting Enzyme Inhibition in Patients With Alport Syndrome Carrying Pathogenic Heterozygous COL4A3,COL4A4 or COL4A5 Mutations

Alport syndrome (AS) is the second most common monogenic cause of end-stage renal failure (ESRF). AS is caused by variants in the COL4A3, COL4A4, and COL4A5 genes, which encode for the a3, a4, and a5 chains of type IV collagen. This trial is a prospective, randomized, controlled and multicenter trial. Mainly to assess the safety and efficacy of ramipril in Alport syndrome patients with variants of COL4A3/COL4A4/COL4A5.

Inclusion Criteria:

1. Age: 30-50 Years;
2. Sex: All;
3. Alport syndrome patients with variants of COL4A3/COL4A4/COL4A5; hematuria or microalbuminuria; eGFR>90 mL/min/1.73m2;
4. Patients with microscopic hematuria only;
5. Patients with microscopic hematuria and microalbuminuria: 30-300mg/24h or urine albumin/creatinine: 30-300mg/g;
6. No angiotensin converting enzyme inhibitor (ACEI) and other renin-angiotensin system inhibitors (including angiotensin II receptor antagonists, etc.) treatment.

Exclusion Criteria:

1. With primary or secondary kidney disease, including IgA nephropathy, membranous nephropathy, lupus nephropathy, benign renal arterioles, etc.;
2. Patients with a history of angioedema;
3. Hypovolemia or hypotension (systolic blood pressure less than 90mmHg and/or diastolic blood pressure less than 60mmHg);
4. Pregnant and lactating women;
5. Patients with bilateral renal artery stenosis or unilateral renal artery stenosis with solitary kidney;
6. Hyperkalemia, blood potassium>5.5mmol/L;
7. Severe aortic stenosis, severe mitral stenosis;
8. Treatment of drug allergy;
9. Hypertension or other diseases that may require treatment with angiotensin-converting enzyme inhibitors;
10. Disagree to participate in this research.