Table 4.C4 deficiencies PatientNr.1. Patient Nr.2 PatientNr.2 PateintNr.4 Sex Female Female Female Female Age 8 54 5 31 Clinical course Transposition of great Rheumatic fever, Septic infection in Meningitis arteries,chronic meningitis,lupus like newborn age Recurrent acute hepatitis with skin disease, pyodermias delayed antibody arthralgias, Lupus formation △pemia of cbronic enthematodes Laboratory CH100 normal c344 C3 normal C3 normal,C4, investigation C3 normal C4 antigen normal C4↓，CHi00 normal CH100 C4 not detecable C4 hemolytic↓ lgG,lgA,lgM normal lgG,IgA,IgM normal lgG↑，lgA,Ana, MBL normla ANCA type p positive ENA SS-B positive ANCA.dsDNA. CH100 IgG kappa positive Cardiolipin antibodies Genetic ASMA I KNM Factor B normal ANCA EI ISA method nositive phenotypisation C4A3AQ0 B2B2 C4A3A3B1BQ0 C4B Q0 C4AQ0C4BQ0 heterozvaous Therapy Interferon A Antimicrobial therapy Corticosteroids, vaccination (Pneumococcus. Meningiciccus,Table 4. C4 deficiencies CH100  Patient Nr. 1. Patient Nr. 2 Patient Nr.2 Pateint Nr.4 Sex Female Female Female Female Age 8 54 5 31 Clinical course Transposition of great arteries, chronic acute hepatitis with delayed antibody formation Rheumatic fever, meningitis, lupus like skin disease, arthralgias, Anemia of chronic infection, recurrent pyodermia Septic infection in newborn age Meningitis Recurrent pyodermias Lupus erythematodes, autoimmune cytopenia Laboratory investigation CH100 normal C3 normal C4 not detecable IgG, IgA, Ana, ANCA, dsDNA, ASMA, LKNM negative HbsAg positive, HbeAg positive C3 C4 antigen normal C4 hemolytic  MBL normla CH100  Factor B normal Nephritic factor negative Facor H normal Factor I normal C3 normal C4, CH100 normal IgG, IgA, IgM normal ANCA type p positive IgG kappa positive ANCA ELISA method normal C3 normal, C4, CH100 , IgG, IgA, IgM normal ENA SS-B positive Cardiolipin antibodies positive Coombs test positive Genetic phenotypisation C4A3AQ0 B2B2 C4A3A3B1BQ0 C4B Q0 heterozygous C4AQ0C4BQ0 Therapy Interferon A Antimicrobial therapy Corticosteroids, vaccination (Pneumococcus, Meningiciccus, Haemophilus)