Inherited factor H deficiency and collagen type III glomerulopathy

Beth A. Vogt, Robert J. Wyatt, Barbara A. Burke, Susan C. Simonton, Clifford Kashtan

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77 Scopus citations

Abstract

A non-immune complex-mediated glomerulonephritis associated with persistent hypocomplementemia occurred in a young boy. Measurement of complement components revealed complete factor H deficiency, inherited as an autosomal recessive trait. Evaluation of the renal lesion revealed extensive deposition of type III collagen suggestive of collagen type III glomerulopathy, a recently identified cause of chronic renal insufficiency in children and adults. This report represents the first association of inherited factor H deficiency with collagen type III glomerulopathy.

Original languageEnglish (US)
Pages (from-to)11-15
Number of pages5
JournalPediatric Nephrology
Volume9
Issue number1
DOIs
StatePublished - Feb 1995

Keywords

  • Collagen type III glomerulopathy
  • Complement
  • Factor H
  • Glomerulonephritis
  • β-1 H globulin

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