COL4A3

Management

AD Alport syndrome. Treatment is started when microalbuminuria (low levels of protein in the urine) are first detected. Treatment is with RAAS blockade with ACE inhibitors (most often ramipril) and sometimes with SGLT2 inhibitors (flozins). This is to minimise the amount of protein in the urine which is toxic to the kidney tissue when it is reabsorbed.  Flozins have not yet been proven safe and effective in children but the trials will conclude late 2025.

AR Alport syndrome. Treatment is started from the time of diagnosis and again is with RAAS blockade (ramipril) and SGLT2 inhibitors (flozins). Flozins have not yet been proven safe and effective in children but the trials will conclude late 2025.

In addition people with AD Alport syndrome or AR Alport syndrome should not smoke, not become overweight or develop diabetes, have well-controlled blood pressure, and exercise regularly.