The three exceptions were a male who presented also with retinal drusen at the age of 10 years and had no hematuria at the last clinical visit, a male with additional nephrotic range proteinuria since the age of 16 years, which persisted at the age of 19 years despite treatment with the maximal dose of angiotensin-converting enzyme inhibitors (ACEI), and a male with confirmed additional sensory hearing loss. The gene discussed is ACE; the disease is retinal drusen.