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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Experimental group | Experimental | Drug: Ramipril The initial dose of ramipril is 2.5 mg /d. The blood pressure, blood potassium and blood creatinine is measured every 1-2 weeks. If the blood pressure is normal, the dose of ramipril is adjusted to 5 mg /d after 2 weeks. If the blood pressure is low, the dose of ramipril is reduced to 1.25 mg /d until the blood pressure becomes normal, otherwise, stop ramipril using. If the blood potassium is high (>5.5mmol/L), the dose of ramipril is reduced to 1.25 mg /d until the blood potassium becomes normal, otherwise, stop ramipril using. If the blood creatinine is higher before therapy (≥30%), the dose of ramipril is reduced to 1.25 mg /d until the blood creatinine becomes normal, otherwise, stop ramipril using. |
|
| Control group | No Intervention | No angiotensin converting enzyme inhibitor (ACEI) and other renin-angiotensin system inhibitors (including angiotensin II receptor antagonists, etc.) treatment. |
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Ramipril | Drug | We use ACEI: ramipril, in this prospective, randomized, controlled and multicenter clinical trial to access the safety and efficacy in Alport syndrome patients carried COL4A3/COL4A4/COL4A5 variants. |
| Measure | Description | Time Frame |
|---|---|---|
| Disease progression time | a) Patients from no proteinuria to microalbuminuria; b) patients from microalbuminuria to dominant proteinuria. | Up to 240 weeks |
| Measure | Description | Time Frame |
|---|---|---|
| 5-year disease progression rate and eGFR slope | 5-year disease progression rate and eGFR slope | Up to 240 weeks |
| Measure | Description | Time Frame |
|---|---|---|
| Number of patients with adverse events | Number of patients with adverse events | Up to 240 weeks |
Inclusion Criteria:
Exclusion Criteria:
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| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Gengru Jiang | Contact | +86-13917983703 | jianggeng-ru@hotmail.com |
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Xinhua Hospital, Shanghai Jiao Tong University School of Medicine | Shanghai | China |
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| ID | Term |
|---|---|
| D009394 | Nephritis, Hereditary |
| ID | Term |
|---|---|
| D014564 | Urogenital Abnormalities |
| D052776 | Female Urogenital Diseases |
| D005261 | Female Urogenital Diseases and Pregnancy Complications |
| D000091642 | Urogenital Diseases |
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| ID | Term |
|---|---|
| D017257 | Ramipril |
| ID | Term |
|---|---|
| D006574 | Heterocyclic Compounds, 2-Ring |
| D000072471 | Heterocyclic Compounds, Fused-Ring |
| D006571 | Heterocyclic Compounds |
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| D009393 | Nephritis |
| D007674 | Kidney Diseases |
| D014570 | Urologic Diseases |
| D052801 | Male Urogenital Diseases |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D003095 | Collagen Diseases |
| D003240 | Connective Tissue Diseases |
| D017437 | Skin and Connective Tissue Diseases |