Medical Genetics Complement disorders Defects of complement components Clq deficiency Combined immunodeficiency: SLE-like syndrome Clrs CIs ,,,+,,,,+ C4 SLE-like syndrome, glomerulonephritis C2 C3 (ACD) Pyogenic infections C7 Neisserial infections C8 C9 +=,++++ None proven Defects of control proteins Cl 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