Over a 31-year period, we have encountered 13 children with a disease entit
y not reported by other clinics that leads to rapidly progressive crescenti
c glomerulonephritis. Gross hematuria, rapidly declining renal function, an
d a serum C3 level at the lower limit of normal or slightly depressed usual
ly characterized the disease onset; hypertension and nephrotic syndrome wer
e absent. Glomerular IgG was absent, but large C3-containing subepithelial
deposits on the paramesangial basement membrane (GBM) were always present.
Because of these deposits and because dense alteration of the GEM was found
in 3 patients, the disease may resemble membranoproliferative glomerulonep
hritis type II, but is distinguishable on other morphological and clinical
grounds. The absence of anti-neutrophil cytoplasmic antibody, tested for in
5 of 13 patients, is one of several ways the disease differs from the pauc
i-immune glomerulonephritis of adults. Clinically and by glomerular morphol
ogy, it also differs from severe poststreptococcal acute glomerulonephritis
. Treatment with high-dose corticosteroids has been highly successful. Beca
use in this series the disease occurred only in children under age 12 years
and the amount of silver-positive mesangial matrix was normal, indicating
absence of mesangial proliferation. it has been designated juvenile acute n
on-proliferative glomerulitis.