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Summary at a Glance  The study revealed that 69% of families with hereditary nephritis that was difficult to diagnose clinicopathologically had heterozygous mutations of COL4A3/A4 (TBMN/ADAS). The finding suggests the importance of genetic testing in appropriate patients.

Autosomal dominant form of type IV collagen nephropathy exists among patients with hereditary nephritis difficult to diagnose clinicopathologically