Familial focal segmental glomerulosclerosis

Int J Pediatr Nephrol. 1983 Dec;4(4):231-4.

Abstract

We have observed the nephrotic syndrome in more than one sibling in three unrelated hispanic families. The histological lesion in the involved children was either focal segmental glomerulosclerosis or IgM nephropathy evolving into focal sclerosis. Tissue typing revealed the presence of HLA DRw8 in six out of eight patients. The frequency of this antigen in our patients, when compared with its frequency in a group of unrelated normal hispanic children was highly significant (p less than 0.0001). Our study suggests that there may be a genetic predilection towards developing focal segmental glomerulosclerosis.

MeSH terms

  • Adolescent
  • Child
  • Genes, MHC Class II*
  • Glomerulonephritis / genetics*
  • Glomerulosclerosis, Focal Segmental / complications
  • Glomerulosclerosis, Focal Segmental / genetics*
  • Hispanic Americans
  • Histocompatibility Antigens Class II / analysis
  • Humans
  • Immune Complex Diseases / complications
  • Immunoglobulin M
  • Nephrotic Syndrome / complications
  • Nephrotic Syndrome / genetics
  • Pedigree

Substances

  • Histocompatibility Antigens Class II
  • Immunoglobulin M