ba0004p31 | (1) | ICCBH2015
De Rechter Stephanie
, Bacchetta Justine
, Dubourg Laurence
, Cochat Pierre
, Van Dyck Mieke
, Evenepoel Pieter
, Levtchenko Elena
, Mekahli Djalila
Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenic cause of renal failure. Data from adult ADPKD population show increased fibroblast growth factor 23 (FGF23) levels while circulating Klotho levels decrease, with a low TmP/GFR even in patients with normal renal function. Moreover, in ADPKD animal models, cyst lining renal cells were demonstrated to produce FGF23, although the animals displayed FGF23 resistance. No data are available in a paediatr...