Insights gained during the last 30 years of research in Alport syndrome have influenced the way potential related kidney donors for Alport patients with end-stage renal disease (ESRD) are assessed. Awareness of Alport syndrome among clinicians and families has increased greatly, so the disease is more likely to be suspected in patients with haematuria and in kindreds with renal disease. Widespread application of electron microscopy, immunohistochemistry and molecular genetics has enhanced diagnostic accuracy, with the result that we are more likely to know which patients and families truly have Alport syndrome. We have learned that Alport syndrome is primarily an X-linked disorder (∼80% of families) and that only ∼10% of affected males represent de novo mutations, meaning that the mother of a boy with Alport syndrome is most likely a heterozygous carrier. Over 95% of heterozygous women with X-linked Alport syndrome (XLAS) exhibit haematuria, so detection of affected women in XLAS kindreds is a straightforward process [1]. Finally, it has been demon