Complement deficiency is associated with an increased prevalence of pyogenic infections and immune complex disease. The spectrum of disease in deficient individuals depends on the stage in the complement system at which the block in activation occurs.
Functions of the proteins that follow in the cascade.Inherited complement deficiencies are classified into two general categories: 1) integral component defects and 2) regulatory component defects.)
Panel test
- Complement activity (CH50) and alternative pathway (AH50)
- Mannose Binding Lectin
- Ani-C1q antibody (IgG)
- C1q Binding assay
- Complement component level
- C1-Esterase inhibitor
References
- Picard C, Gaspar B, Al-Herz W, Bousfiha A, Casanova J, Chatila T, Crow YJ, Cunningham-Rundles C, Etzioni A, Franco JL, Holland SM, Klein C, Morio T, Ochs HD, Oksenhendler E, Puck J, Tang ML, Tangye SG, Torgerson TR, Sullivan KE. International Union of Immunological Societies: 2017 Primary Immunodeficiency Diseases Committee Report on Inborn Errors of Immunity. J Clin Immunol. 2018; 38(1): 96-128. PubMed
- Bonilla FA, Bernstein L, Khan DA, Ballas ZK, Chinen J, Frank MM, Kobrynski LJ, Levinson AI, Mazer B, Nelson RP, Orange JS, Routes JM, Shearer WT, Sorensen RU, American Academy of Allergy, Asthma and Immunology, American College of Allergy, Asthma and Immunology, Joint Council of Allergy, Asthma and Immunology. Practice parameter for the diagnosis and management of primary immunodeficiency. Ann Allergy Asthma Immunol. 2005; 94(5 Suppl 1): S1-63. PubMed
