Fibronectin glomerulopathy

**Definition:** Fibronectin glomerulopathy is a rare hereditary kidney disease in which fibronectin (FN1) deposits are seen in the mesangium and subendothelial space. The clinical picture is characterised by proteinuria, type IV renal tubular acidosis, microscopic haematuria and hypertension that may lead to end-stage renal failure in the second to sixth decade of life. This disease may be associated with mutations in the FN1 gene.