Abstract:
:Complement factor I (CFI) deficiency is typically associated to recurrent infections with encapsulated microorganisms and, less commonly, to autoimmunity. We report a 53-years old male who, in a routine control for non-alcoholic fatty liver disease, presented a flat beta-2 fraction at the capillary protein electropherogram. Patient's clinical records included multiple oropharyngeal infections since infancy and an episode of invasive meningococcal infection. Complement studies revealed reduced C3, low classical pathway activation and undetectable Factor I. CFI gene sequencing showed a novel inherited homozygous deletion of 5 nucleotides in exon 12, causing a frameshift leading to a truncated protein. This study points out that capillary protein electrophoresis can alert of possible states of low C3, which, once confirmed and common causes ruled out, can lead to CFI and other complement deficiency diagnosis. This is important since they constitute a still underestimated risk of invasive meningococcemia that can be greatly reduced by vaccination.
journal_name
Immunol Lettjournal_title
Immunology lettersauthors
Franco-Jarava C,Colobran R,Mestre-Torres J,Vargas V,Pujol-Borrell R,Hernández-González Mdoi
10.1016/j.imlet.2016.04.011subject
Has Abstractpub_date
2016-06-01 00:00:00pages
19-22eissn
0165-2478issn
1879-0542pii
S0165-2478(16)30062-1journal_volume
174pub_type
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