We reviewed the clinical and renal biopsy findings in 322 children pre
senting during the years 1975-1996 with recurrent macro- or continuous
microscopic haematuria persisting for greater than or equal to 6 mont
hs, in whom non-glomerular causes were excluded. Family involvement wa
s documented for first-degree relatives. All biopsies were examined by
light microscopy, 317 by electron microscopy and 315 by immunofluores
cence. Biopsies were classified as IgA nephropathy (78), Alport nephro
pathy (86), thin basement membrane nephropathy (TMN) (50), miscellaneo
us glomerulonephritis (32), hilar vasculopathy (28) and normal glomeru
li (48). Although microscopic haematuria alone was more frequent in Al
port nephropathy and TMN, the pattern of haematuria in individual pati
ents did not predict histology. Of patients with familial haematuria,
79% of biopsies showed either Alport nephropathy or TMN. Hilar vasculo
pathy was observed both in isolation and in all abnormal histological
categories.