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.
Publication types
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Case Reports
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Research Support, U.S. Gov't, P.H.S.
MeSH terms
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Blood Coagulation Disorders / genetics
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Blood Coagulation Disorders / pathology
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Blood Coagulation Factors / metabolism
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Child
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Collagen / genetics*
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Collagen / metabolism
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Collagen Diseases / genetics*
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Collagen Diseases / metabolism
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Collagen Diseases / pathology
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Complement Factor H / analysis
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Complement Factor H / deficiency*
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Complement System Proteins / metabolism
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Glomerulonephritis / genetics*
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Glomerulonephritis / metabolism
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Glomerulonephritis / pathology
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Humans
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Kidney / pathology
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Male
Substances
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Blood Coagulation Factors
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CFH protein, human
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Complement Factor H
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Collagen
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Complement System Proteins