Medical Genetics Complement disorders Defects of complement components C1q deficiency……… +4, Combined immunodeficiency: SLE-like syndrome Clrs CIs C4 SLE-like syndrome, glomerulonephritis C2 C5 (ACD).......Pyogenic infections C6 C7 Neisserial infections C8 C9 +++4,++““4““ None proven Defects of control proteins CI inhibitor deficiency (AD) Angioedema, SLE Factor I(C3b inactivator) deficiency (ACD Pyogenic infections Factor H deficiency (ACD) Hemolytic-uremic syndrome, glomerulo nephritis Factor d deficiency(ACD Pyogenic infections Properdin deficiency (XL) Neisserial infections XL=X-linked: Ig=immunoglobulin; AR= autosomal recessive: ACD= autosomal codominant: AD autosomal dominantMedical Genetics