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.
Similar content being viewed by others
References
Thompson R, Winterborn M (1981) Hypocomplementemia due to a genetic deficiency of β1H globulin. Clin Exp Immunol 46: 110–119
Levy M, Halbwachs-Mecarelli L, Gubler M-C, Kohout G, Bensenouci A, Niaudet P, Hauptmann G, Lesavre P (1986) Factor H deficiency in two brothers with atypical dense intramembranous deposit disease. Kidney Int 30: 949–956
Lopez-Larrea C, Dieguez M, Enguix A, Dominguez O, Marin B, Gomez E (1987) A familial deficiency of complement factor H. Biochem Soc Trans 15: 648–649
Brai M, Misiano G, Maringhini S, Cutaja I, Hauptmann G (1988) Combined homozygous factor H and heterozygous C2 deficiency in an Italian family. J Clin Immunol 8: 50–56
Wyatt R, Forristal J, Davis C, Coleman T, West C (1980) Control of serum C3 levels by B-1 H and C3b inactivator. J Lab Clin Med 95: 905–917
Kim Y, Vernier R, Fish A, Michael A (1979) Immunofluorescence studies of dense deposit disease: the presence of railroad tracks and mesangial rings. Lab Invest 40: 474–480
Marinozzi V (1963) The role of fixation in electron staining. J R Microsc Soc 81: 141–154
Nilsson U, Muller-Eberhard H (1965) Isolation of B-IF-globulin from human serum and its characterization as the fifth component of complement. J Exp Med 146: 257–270
Vik D, Munoz-Canoves P, Chaplin D, Tack B (1989) Factor H. Curr Top Microbiol Immunol 153: 147–162
Nielsen H, Christensen K, Koch C, Thomsen B, Heegaard N, Tranum-Jensen J (1989) Hereditary, complete deficiency of complement factor H associated with recurrent meningococcal disease. Scand J Immunol 30: 711–718
Fijen C, Kuijper E, Holdrinet A, Daha M, Dankert J (1992) Factor H deficiency in a Dutch family. Immunobiology 184: 427
Wyatt R, Julian B, Weinstein A, Rothfield N, McLean R (1982) Partial H (β1H) deficiency and glomerulonephritis in two families. J Clin Immunol 2: 110–117
Gubler M, Dommergues J, Foulard M, Bensman A, Leroy J, Broyer M, Habib R (1993) Collagen type III glomerulopathy: a new type of hereditary nephropathy. Pediatr Nephrol 7: 354–360
Ikeda K, Yokoyama H, Tomosugi N, Kida H, Ooshima A, Kobayashi K (1990) Primary glomerular fibrosis: a new nephropathy caused by diffuse intra-glomerular increase in atypical type III collagen fibers. Clin Nephrol 33: 155–159
Imbasciati E, Gherardi G, Morozumi K, Gudat F, Epper R, Basler V, Mihatsch M (1991) Collagen type III glomerulopathy: a new idiopathic glomerular disease. Am J Nephrol 11: 422–429
Yoshida F, Yuzawa Y, Shigematsu H, Ito A, Yamazaki C, Yoshioka K, Ooshima A, Matsuo S (1993) Nephrotic syndrome with massive accumulation of type I and type III collagen in the glomeruli. Intern Med 32: 171–176
Mizuiri S, Hasegawa A, Kikuchi A, Amagasaki Y, Nakamura N, Sakaguchi H (1993) A case of collagenofibrotic glomerulopathy associated with hepatic perisinusoidal fibrosis. Nephron 63: 183–187
Yoshioka K, Takemura T, Tohda M, Akano N, Miyamoto H, Ooshima A, Maki S (1989) Glomerular localization of type III collagen in human kidney disease. Kidney Int 35: 1203–1211
Meyrier A, Rizzo R, Gubler M (1990) The nail-patella syndrome. A review. J Nephrol 2: 133–140
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
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
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00858956