Inherited C8 beta subunit deficiency in a patient with recurrent meningococcal infections: in vivo functional kinetic analysis of C8.
A 16 year old with recurrent meningococcal infections is reported. Absence of haemolytic activity in both the classical and alternative pathways resulted from an absence of functional C8. Addition of functional C8 restored hemolytic activity. Antigenically deficient C8 was present in the serum and isoelectric focusing of serum confirmed the absence of the C8 beta chain. Following the infusion of fresh frozen plasma, we followed the decay in C8 functional activity as well as total haemolytic activity. C8 activity peaked at about 3 h with a half-life survival estimated to be 28 h. The kinetics of total haemolytic activity showed a slower decay with an exponential decline over 72 h and a half-life of 55 h. Fresh frozen plasma may be of value in the treatment of patients with C8 deficiency and acute Neisserial infections.
D000293:Adolescent; D003167:Complement Activation; D003185:Complement C8 / Q000172:deficiency* / Q000276:immunology; D005260:Female; D006680:HLA Antigens / Q000032:analysis; D006207:Half-Life; D006461:Hemolysis; D006801:Humans; D005779:Immunodiffusion; D007700:Kinetics; D008297:Male; D008589:Meningococcal Infections / Q000276:immunology*; D011233:Precipitin Tests; D012008:Recurrence
D003185:Complement C8; D006680:HLA Antigens