Acute non-proliferative glomerulitis: a cause of renal failure unique to children.
Over a 31-year period, we have encountered 13 children with a disease entity not reported by other clinics that leads to rapidly progressive crescentic glomerulonephritis. Gross hematuria, rapidly declining renal function, and a serum C3 level at the lower limit of normal or slightly depressed usually characterized the disease onset; hypertension and nephrotic syndrome were 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 GBM was found in 3 patients, the disease may resemble membranoproliferative glomerulonephritis 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 pauci-immune glomerulonephritis of adults. Clinically and by glomerular morphology, it also differs from severe poststreptococcal acute glomerulonephritis. Treatment with high-dose corticosteroids has been highly successful. Because 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 non-proliferative glomerulitis.
West
C D
CD
Children's Hospital Research Foundation and the Department of Pediatrics, University of Cincinnati College of Medicine, Ohio, USA. howajo@chmcc.org
McAdams
A J
AJ
Witte
D P
DP
D000208:Acute Disease; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D003404:Creatinine / Q000097:blood; D018450:Disease Progression; D005921:Glomerulonephritis / Q000150:complications* / Q000175:diagnosis* / Q000503:physiopathology; D006417:Hematuria; D006801:Humans; D007668:Kidney / Q000473:pathology / Q000648:ultrastructure; D007676:Kidney Failure, Chronic / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D012008:Recurrence; D012075:Remission, Spontaneous; D012189:Retrospective Studies
D003404:Creatinine