title: [Hereditary complement deficiencies].
abstract: Complement deficiencies of all nine C-components have been observed. Hereditary defects of early components of the classical pathway - C1, C4, C2 - are often associated with diseases of the immuncomplex-type especially with systemic lupus erythematosus, dermatomysitis, vasculitis and nephritis. Deficiencies of C3 and C3b inactivator are linked to severe and recurrent bacterial infections. Patients with hereditary defects of the so-called late components, C5-C9, show increased susceptibility to recurrent disseminated infections by neisseria gonorrhoeae and meningitidis. The most frequent of the defects of the complement system is the hereditary deficiency of C1-inactivator which is associated with hereditary angioneurotic edema. In this paper the C-defects and their genetics are described and possible pathomechanisms are discussed.
mesh_terms: D003166:Complement Activating Enzymes / Q000172:deficiency; D003167:Complement Activation; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency; D015922:Complement C1q; D015923:Complement C1r; D003173:Complement C1s; D003175:Complement C2 / Q000172:deficiency; D003176:Complement C3 / Q000172:deficiency; D003180:Complement C3b Inactivator Proteins / Q000172:deficiency; D003181:Complement C4 / Q000172:deficiency; D003182:Complement C5 / Q000172:deficiency; D003186:Complement C9 / Q000172:deficiency; D003165:Complement System Proteins / Q000172:deficiency* / Q000502:physiology; D005260:Female; D005817:Genetic Counseling; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000235:genetics*; D008297:Male
chemical_list: D003174:Complement C1 Inactivator Proteins; D003175:Complement C2; D003176:Complement C3; D003180:Complement C3b Inactivator Proteins; D003181:Complement C4; D003182:Complement C5; D003186:Complement C9; D015922:Complement C1q; D003165:Complement System Proteins; D003166:Complement Activating Enzymes; D015923:Complement C1r; D003173:Complement C1s
vernacular_title: Hereditäre Komplmentdefekte.