Paucity of minimal-change lesion in children with early frequently relapsing steroid-responsive nephrotic syndrome

Am J Nephrol. 1987;7(1):13-7. doi: 10.1159/000167422.

Abstract

It is widely believed that the most common morphological lesion in children with the idiopathic nephrotic syndrome who manifest a frequently relapsing steroid-responsive course is the minimal-change lesion. However, there are no prospective renal biopsy studies in such patients to substantiate this assertion. We performed a renal biopsy in all children with early frequently relapsing steroid-responsive nephrotic syndrome during the years 1980-1984. In 16 affected children, only 4 (25%) had minimal-change lesion, 7 had IgM nephropathy, 3 had diffuse mesangial hypercellularity, and 2 had focal segmental glomerulosclerosis. Fourteen of these patients have required immunosuppressive therapy with cyclophosphamide. Long-term follow-up revealed that 10 patients have remained protein free, 4 have persistent proteinuria despite cyclophosphamide therapy, 1 had progressed to end-stage renal disease, and 1 is lost to follow-up. On the basis of these findings, we recommend that all children with nephrotic syndrome and an early frequently relapsing steroid-responsive course undergo a prompt renal biopsy. Such patients constitute a high-risk group with a spectrum of renal histopathological lesions characterized by an unpredictable response to therapy and an unfavorable prognosis.

MeSH terms

  • Child
  • Child, Preschool
  • Female
  • Humans
  • Kidney / pathology*
  • Kidney / ultrastructure
  • Male
  • Nephrosis, Lipoid / complications
  • Nephrosis, Lipoid / pathology*
  • Nephrotic Syndrome / complications
  • Nephrotic Syndrome / pathology*
  • Prognosis
  • Prospective Studies
  • Recurrence