Crescentic glomerulonephritis in children
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Data on patients with crescentic glomerulonephritis (>50% glomeruli with crescents), referred to the Hospital for Sick Children during the past 13 years, were reviewed. Thirty patients (13 male, 17 female) aged 3.7–15.7 years (mean 9.5) were evaluated. Initial clinical features included: oedema (24/30), hypertension (19/30), gross haematuria (15/30), oliguria (15/30) and a decreased glomerular filtration rate (GFR<30 ml/min per 1.73 m2) (22/30). Henoch-Schönlein purpura was present in 9 patients, microscopic polyarteritis in 3, polyarteritis nodosa in 1, Wegener's granulomatosis in 1, systemic lupus erythematosus in 1, post-streptococcal glomerulonephritis in 2, mesangiocapillary glomerulonephritis in 7, anti-glomerular basement membrane glomerulonephritis in 2, and 4 were idiopathic. In 10 patients 50%–79% of glomeruli were affected by crescentic changes (group 1) and in the remaining 20, 80% or more (group 2). The crescents were cellular, fibrocellular or fibrous, and the degree of sclerosis was assessed. Patients in both groups were treated with plasma exchange, corticosteroids, anticoagulants, cyclophosphamide and azathioprine in different combinations. On follow-up, 3 patients were dead, 1 was lost to follow-up, 12 were on dialysis/transplant programmes, 4 had a GFR of less than 30 and 10 a GFR of more than 30 ml/min per 1.73 m2. In our experience, 50% progressed to end-stage renal failure. The interval between disease onset and start of treatment was a prognostic factor for outcome. Fibrous crescents were associated with a worse outcome than fibrocellular crescents (P<0.05). Outcome was not, however, related to the percentage of glomeruli affected (P>0.05). Although the effectiveness of the individual components of the treatment regimens used was difficult to assess, one-third of patients at the latest follow-up had a GFR of more than 30 ml/min per 1.73 m2.
Key wordsCrescentic glomerulonephritis Rapidly progressive glomerulonephritis
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- 1.Southwest Pediatric Nephrology Study Group (1985) A clinicopathologic study of crescentic glomerulonephritis in 50 children. Kidney Int 27: 450–458Google Scholar
- 2.Niaudet P, Levy M (1983) Glomérulonéphrites à croissants diffus. In: Royer P, Habib R, Mathieu H, Broyer M (eds) Néphrologie Pédiatrique, 3rd edn. Flammarion, Paris, pp 381–394Google Scholar
- 5.Heptinstall RH (1983) Crescentic glomerulonephritis. In: Heptinstall RH (ed) Pathology of the kidney, 3rd edn. Little Brown, Boston, pp 443–447Google Scholar
- 7.Robson AM, Rose GM, Cole BR, Ingelfinger JR (1981) The treatment of severe glomerulopathies in children with intravenous methylprednisolone pulses. Proceedings of the 8th International Congress on Nephrology, Athens S-6 Karger, Basel: 305–311Google Scholar
- 8.National Heart, Lung and Blood Institute, Bethesda, Maryland (1987) Report of the second task force on blood pressure control in children. Pediatrics 79: 1–25Google Scholar
- 9.Savage CO, Jones S, Winearls CG, Marshall PD, Lockwood CM (1987) Prospective study of radioimmunoassay for antibodies against neutrophil cytoplasm in diagnosis of systemic vasculitis. Lancet I: 1389–1393Google Scholar
- 14.Stevens ME, McConnell M, Bone JM (1982) Aggressive treatment with pulse methylprednisolone or plasma exchange is justified in rapidly progressive glomerulonephritis. Proc Eur Dial Transplant Assoc 19: 724–731Google Scholar
- 15.Couser WG (1988) Rapidly progressive glomerulonephritis: classification, pathogenetic mechanisms and therapy. Am J Kidney Dis 6: 449–464Google Scholar
- 16.Lockwood CM, Rees AJ, Pearson TA, Evans DJ, Peters DK, Wilson CB (1976) Immunosuppression and plasma exchange in the treatment of Goodpasture's syndrome. Lancet I: 711–715Google Scholar
- 17.Lockwood CM, Rees AJ, Pinching AJ, Pussell B, Sweny P, Uff J, Peters DK (1977) Plasma exchange and immunosuppression in the treatment of fulminating immune-complex mediated crescentic nephritis. Lancet I: 63–67Google Scholar
- 19.Glockner WH, Sieberth HG, Wichmann HE, Backes E, Bambauer R, Boesken WH, Bohle A, Daul A, Graben N, Keller F, Klehr HU, Kohler H, Metz U, Schultz W, Thoenes W, Vlaho M (1988) Plasma exchange and immunosuppression in rapidly progressive glomerulonephritis: a controlled, multi-center study. Clin Nephrol 1: 1–8Google Scholar