Besides renal manifestations, these diseases can present cochlear and ocular abnormalities (2). However, the hallmark of Collagen IV-related nephropathies is hematuria. Proteinuria is detected during life, being a marker of disease progression. All males with XLAS develop proteinuria and, eventually, progressive renal insufficiency, which leads to end-stage renal disease (ESRD). Overall, an estimated 60% reach ESRD by age 30 years, and 90% by age 40 years (2). Approximately 12% of females with XLAS develop ESRD before age 40 years, increasing to 30% by age 60 years (3). Most individuals with ARAS develop significant proteinuria in late childhood or adolescence and ESRD before age 30 years. Progression to ESRD is slower in individuals with ADAS. Similarly, TBMN is characterized clinically by persistent microhematuria often observed in childhood. TBMN is rarely associated with extrarenal abnormalities and proteinuria. Hypertension and progression to ESRD are unusual (4).