Abstract
We have shown that children with aCAH have genetically determined low C4 levels but it is not known whether C4 function is also impaired. In the present study we measured haemolytic function of C4 and its concentration in 20 children with aCAH(15 female, median age 13½yrs, range 7-18yrs) and in 19 sex and age matched normal controls. C4 function was measured by a radial haemolytic assay, using C4 deficient guinea-pig serum, the concentration by laser nephelometry. A significant reduction of C4 levels was observed in patients [median 0.15g/l, range 0.04-0.485 g/1] compared to healthy children [0.263g/l, 0.153-0.520g/l, p < 0.002] confirming our previous data. C4 function in 16 normal children was 50% (25-100%) but in 3 was very low (4%, 4%, 122). In patients C4 function was 12%(0-67%), significantly reduced when compared to all controls. (41.5%, 4-100%, p< 0.001). There was no significant correlation between function and concentration values. Such discrepancy between function and concentration could be explained by the marked genetic polymorphism of C4. These data show that C4 function is impaired in aCAH. C4 has a key role in virus neutralization and impairment of its function might enable a virus to initiate the autoimmune process leading to CAH.
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Mieli-Vergani, G., Mowat, A., Vergani, D. et al. 62. LOW C4 HAEMOLYTIC FUNCTION IN AUTOIMMUNE CHRONIC ACTIVE HEPATITIS (aCAH)OF CHILDHOOD. Pediatr Res 22, 106 (1987). https://doi.org/10.1203/00006450-198707000-00083
Issue Date:
DOI: https://doi.org/10.1203/00006450-198707000-00083