C8 β subunit deficiency in a patient with recurrent Neisserial infections

Luigi Fontana, Francesco Tedesco, Caterina De Carolis, Lucia Roncelli, Antonella Teggi, Amerigo Paffetti, Roberto Perricone

Research output: Contribution to journalArticlepeer-review

Abstract

A 15-year-old woman with a history of recurrent episodes of meningococcal infections was admitted to our hospital with signs and symptoms indicating a meningeal inflammation. Since in the last few years some of the patients affected by recurrent meningococcal infections have been recognized to have selective complement deficiencies, the patient's serum was studied for determining the complement function. C8 was found to be present only in traces, with a pattern of partial identity as compared with that of the normal human serum. Moreover, total hemolytic complement was undetectable and could be completely restored with purified C8, but not with other complement components; thus, we concluded for the presence of a C8 deficiency state. Further reconstitution experiments carried out with sera having selective deficiencies of either C8 β or C8 α-γ subunits allowed us to recognize the presence of a dysfunctional C8 molecule lacking the β chain, but possessing the α-γ subunit. The clinical history of the patient, characterized by recurrent meningococcal infections, further supports the current concept of an increased susceptibility of the C8 β-deficient patients to Neisseria meningitidis infections.

Original languageEnglish
Pages (from-to)18-25
Number of pages8
JournalLa Ricerca in Clinica e in Laboratorio
Volume17
Issue number1
DOIs
Publication statusPublished - Jan 1987

Keywords

  • C8 α-γ subunit deficiency
  • C8 β subunit deficiency
  • Complement
  • Neisseria gonorrhoeae
  • Neisseria meningitidis

ASJC Scopus subject areas

  • Clinical Biochemistry

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