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Inherited factor H deficiency and collagen type III glomerulopathy

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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.

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Vogt, B.A., Wyatt, R.J., Burke, B.A. et al. Inherited factor H deficiency and collagen type III glomerulopathy. Pediatr Nephrol 9, 11–15 (1995). https://doi.org/10.1007/BF00858956

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  • DOI: https://doi.org/10.1007/BF00858956

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