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• W2061665713 abstract "This report provides a description of 3 patients with chronic glomerulonephritis who present unique serum complement system deficiencies associated with different kidney histopathology and immunopathology. One patient has a severe deficiency of hemolytic C1 predominantly due to diminished C1r function; this abnormality is associated with the end stage of glomerulonephritis. A second patient has a selective deficiency of hemolytic C2 function associated with a membranous glomerulopathy. The third patient has hereditary angioedema with decreased levels of hemolytic C4 and C2; this patient's glomeruli show basement membrane thickening, centrolobular and glomerular hyalinization, mesangial proliferation, and interstitial round cell infiltration similar to that seen in patients with the C3-deficient form of chronic glomerulonephritis in children. The possible basis for the deficiencies and the questions that these natural experiments raise concerning the direct or indirect participation of the complement system in immune injury to the kidney are discussed. This report provides a description of 3 patients with chronic glomerulonephritis who present unique serum complement system deficiencies associated with different kidney histopathology and immunopathology. One patient has a severe deficiency of hemolytic C1 predominantly due to diminished C1r function; this abnormality is associated with the end stage of glomerulonephritis. A second patient has a selective deficiency of hemolytic C2 function associated with a membranous glomerulopathy. The third patient has hereditary angioedema with decreased levels of hemolytic C4 and C2; this patient's glomeruli show basement membrane thickening, centrolobular and glomerular hyalinization, mesangial proliferation, and interstitial round cell infiltration similar to that seen in patients with the C3-deficient form of chronic glomerulonephritis in children. The possible basis for the deficiencies and the questions that these natural experiments raise concerning the direct or indirect participation of the complement system in immune injury to the kidney are discussed." @default.
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• W2061665713 date "1971-01-01" @default.
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• W2061665713 title "The complement system in chronic glomerulonephritis: Three newly associated aberrations" @default.
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• W2061665713 doi "https://doi.org/10.1016/s0022-3476(71)80261-5" @default.
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