C5 Deficiency and Meningitis in a Swiss Family
- 1 March 1988
- journal article
- research article
- Published by American Medical Association (AMA) in Archives of internal medicine (1960)
- Vol. 148 (3) , 754
- https://doi.org/10.1001/archinte.1988.00380030260047
Abstract
To the Editor. —Inherited deficiencies of late complement components are rare and are associated with an increased susceptibility to a single episode or to recurrent neisserial infections and, less frequently, with autoimmune disease.1 To the best of our knowledge only 19 homozygous C5-deficient patients have been described so far.1-5 We report preliminary data of two further cases of a selective C5 deficiency (C5D) in a Swiss family, each revealed by the occurrence of meningitis (Neisseria meningitidis, group C and B, respectively). In the two subjects, C5 was not detectable by radial immunodiffusion (RID) using monospecific antisera. In both patients, sequential titration of hemolytic complement by sensitized sheep red blood cells revealed levels of only 1% of normal. In contrast, the functional alternative pathway was only partially affected (between 50% and 65% of the normal mean) as titrated by lysis of rabbit erythrocytes at physiologic ionic strength in theThis publication has 3 references indexed in Scilit:
- Complement Deficiency and Sporadic Meningococcal DiseaseNew England Journal of Medicine, 1983
- Epidemic Meningococcal Disease: Synthesis of a Hypothetical Immunoepidemiologic ModelClinical Infectious Diseases, 1982
- Hereditary deficiency of the fifth component of complement in man. I. Clinical, immunochemical, and family studies.Journal of Clinical Investigation, 1976