C8 β subunit deficiency in a patient with recurrent Neisserial infections

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