Crescentic glomerulonephritis in children

Pediatr Nephrol. 1992 May;6(3):231-5. doi: 10.1007/BF00878354.

Abstract

Data on patients with crescentic glomerulonephritis (greater than 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 less than 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 less than 0.05). Outcome was not, however, related to the percentage of glomeruli affected (P greater than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adrenal Cortex Hormones / therapeutic use
  • Anticoagulants / therapeutic use
  • Azathioprine / therapeutic use
  • Child
  • Child, Preschool
  • Cyclophosphamide / therapeutic use
  • Female
  • Glomerular Filtration Rate / physiology
  • Glomerulonephritis / complications
  • Glomerulonephritis / drug therapy
  • Glomerulonephritis / pathology*
  • Glomerulonephritis, Membranoproliferative / complications
  • Glomerulonephritis, Membranoproliferative / drug therapy
  • Glomerulonephritis, Membranoproliferative / pathology
  • Granulomatosis with Polyangiitis / complications
  • Granulomatosis with Polyangiitis / pathology
  • Granulomatosis with Polyangiitis / physiopathology
  • Hematuria / etiology
  • Hematuria / physiopathology
  • Humans
  • Hypertension / etiology
  • Hypertension / physiopathology
  • IgA Vasculitis / complications
  • IgA Vasculitis / drug therapy
  • IgA Vasculitis / pathology
  • Kidney / drug effects
  • Kidney / pathology
  • Kidney / physiopathology
  • London
  • Lupus Erythematosus, Systemic / complications
  • Lupus Erythematosus, Systemic / pathology
  • Lupus Erythematosus, Systemic / physiopathology
  • Male
  • Polyarteritis Nodosa / complications
  • Polyarteritis Nodosa / pathology
  • Polyarteritis Nodosa / physiopathology
  • Prognosis
  • Vasculitis / complications
  • Vasculitis / drug therapy
  • Vasculitis / pathology

Substances

  • Adrenal Cortex Hormones
  • Anticoagulants
  • Cyclophosphamide
  • Azathioprine