RESUMEN
PURPOSE: Alport's syndrome is a rare but important cause of renal failure. It is characterized by Type IV collagen mutations resulting in connective tissue disorders and renal and cochlear dysfunction. Vascular basement membrane also contains collagen IV and the effect on arteriovenous fistulas (AVFs) is not reported. Anecdotally, we observed a high rate of aneurysm formation in Arteriovenous fistulas (AVF) of patients with Alport's and sought to determine whether this was the case within our population. METHODS: All patients with a diagnosis of Alport's were identified from a contemporaneously maintained database. AVFs formed in patients with Alport's were identified to define the incidence of aneurysms in this group. RESULTS: A total of 40 patients with a diagnosis of Alport's were identified. Of these, 20 patients had undergone AVF formation, the remainder opting for CAPD as renal replacement or had undergone pre-emptive transplantation. Of the 20 patients identified, 11 had an AVF and of these the rate of aneurysm formation was high (55%). CONCLUSIONS: While this finding of high rate of aneurysmal AVF in Alport's patients is a purely observational finding within our population further population study would be extremely interesting and could support enhanced surveillance or alternative dialysis modalities in Alport's syndrome patients.
