Recurrent haemolytic uraemic syndrome and acquired hypomorphic variant of the third component of complement View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

1990-11

AUTHORS

A. M. Roodhooft, R. H. McLean, E. Elst, K. J. Van Acker

ABSTRACT

In a girl with recurrent haemolytic uraemic syndrome (HUS), persistently low serum levels of C3 were found. Analysis of complement phenotype revealed a hypomorphic variant of C3 Fast in the patient (C3fS) and a normal heterozygous pattern in both parents and the brother (C3FS). Other complement aberrations in the patient were: the presence of a null gene for C4A and C4B and low serum levels of factor H. The father also had partial factor H deficiency. It is hypothesized that the hypomorphic C3 variant may predispose to recurrent HUS. In the acquired forms the role of uraemia in alteration of C3F should be considered. More... »

PAGES

597-599

References to SciGraph publications

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/bf00858631

DOI

http://dx.doi.org/10.1007/bf00858631

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1008697781

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/2088459


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