Abstract
C3 nephritic factor (NEF) has been found in 3 siblings presenting different (or none) degrees of renal disease. Other relatives, including their dead father, suffered from a renal illness. In 2 of the siblings, NEF activity was restricted to IgG1 and IgG3 subclasses. Familial NEF incidence and a shared C3 allotype and a common HLA haplotype including BfS alleles for the 3 NEF-positive siblings suggest that at least in our cases genetical factors may be involved in NEF generation.
Publication types
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Case Reports
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Research Support, Non-U.S. Gov't
MeSH terms
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Adolescent
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Adult
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Complement C3 / analysis
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Complement C3 / genetics
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Complement C3 Nephritic Factor / analysis
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Complement C3 Nephritic Factor / genetics*
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Complement C4 / analysis
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Female
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Glomerulonephritis / blood
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Glomerulonephritis / genetics*
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Glomerulonephritis / immunology
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HLA Antigens / analysis
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Humans
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Immunoglobulin G / analysis
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Immunoglobulin G / classification
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Male
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Middle Aged
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Phenotype
Substances
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Complement C3
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Complement C3 Nephritic Factor
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Complement C4
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HLA Antigens
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Immunoglobulin G