Complement Deficiencies: Multimedia

References

1. Botto M, Kirschfink M, Macor P, Pickering MC, Wurzner R, Tedesco F. Complement in human diseases: Lessons from complement deficiencies. Mol Immunol. Sep 2009;46(14):2774-83. [Medline].

2. Arnold DF, Roberts AG, Thomas A, Ferry B, Morgan BP, Chapel H. A novel mutation in a patient with a deficiency of the eighth component of complement associated with recurrent meningococcal meningitis. J Clin Immunol. Sep 2009;29(5):691-5. [Medline].

3. Sonea MJ, Moroz BE, Reece ER. Leiner's disease associated with diminished third component of complement. Pediatr Dermatol. Aug 1987;4(2):105-7. [Medline].

4. Goodyear HM, Harper JI. Leiner's disease associated with metabolic acidosis. Clin Exp Dermatol. Sep 1989;14(5):364-6. [Medline].

5. Peerschke EI, Yin W, Alpert DR, Roubey RA, Salmon JE, Ghebrehiwet B. Serum complement activation on heterologous platelets is associated with arterial thrombosis in patients with systemic lupus erythematosus and antiphospholipid antibodies. Lupus. May 2009;18(6):530-8. [Medline].

6. Janzi M, Sjoberg R, Wan J, et al. Screening for C3 deficiency in newborns using microarrays. PLoS One. 2009;4(4):e5321. [Medline].

7. Beynon HL, Davies KA, Haskard DO, Walport MJ. Erythrocyte complement receptor type 1 and interactions between immune complexes, neutrophils, and endothelium. J Immunol. Oct 1 1994;153(7):3160-7. [Medline].

8. Brandtzaeg P, Mollnes TE, Kierulf P. Complement activation and endotoxin levels in systemic meningococcal disease. J Infect Dis. Jul 1989;160(1):58-65. [Medline].

9. Carroll MC. The role of complement in B cell activation and tolerance. Adv Immunol. 2000;74:61-88. [Medline].

10. Davies KA, Erlendsson K, Beynon HL, et al. Splenic uptake of immune complexes in man is complement-dependent. J Immunol. Oct 1 1993;151(7):3866-73. [Medline].

11. Davies KA, Peters AM, Beynon HL, Walport MJ. Immune complex processing in patients with systemic lupus erythematosus. In vivo imaging and clearance studies. J Clin Invest. Nov 1992;90(5):2075-83. [Medline].

12. Dempsey PW, Allison ME, Akkaraju S, et al. C3d of complement as a molecular adjuvant: bridging innate and acquired immunity. Science. Jan 19 1996;271(5247):348-50. [Medline].

13. Densen P, Weiler JM, Griffiss JM, Hoffmann LG. Familial properdin deficiency and fatal meningococcemia. Correction of the bactericidal defect by vaccination. N Engl J Med. Apr 9 1987;316(15):922-6. [Medline].

14. Doody GM, Dempsey PW, Fearon DT. Activation of B lymphocytes: integrating signals from CD19, CD22 and Fc gamma RIIb1. Curr Opin Immunol. Jun 1996;8(3):378-82. [Medline].

15. Fauci A, Braunwald E, Isselbacher KJ. Meningococcal Infections. In: Fauci A, Braunwald E, Isselbacher KJ, Wilson JD, Martin JB, Kasper DL, Hauser SL, Longo DL, Harrison TR. Harrison's Principles of Internal Medicine. 14^th ed. New York, NY: McGraw-Hill; 1998:910-5.

16. Figueroa JE, Densen P. Infectious diseases associated with complement deficiencies. Clin Microbiol Rev. Jul 1991;4(3):359-95. [Medline].

17. Frank MM. Complement deficiencies. Pediatr Clin North Am. Dec 2000;47(6):1339-54. [Medline].

18. Girardin E, Grau GE, Dayer JM, et al. Tumor necrosis factor and interleukin-1 in the serum of children with severe infectious purpura. N Engl J Med. Aug 18 1988;319(7):397-400. [Medline].

19. Goodnow CC. Pathways for self-tolerance and the treatment of autoimmune diseases. Lancet. Jun 30 2001;357(9274):2115-21. [Medline].

20. Kang HJ, Kim HS, Lee YK, Cho HC. High incidence of complement C9 deficiency in Koreans. Ann Clin Lab Sci. 2005;35(2):144-8. [Medline].

21. Kuby J. The Complement System. In: Immunology. 3^rd ed. New York, NY: WH Freeman; 1997:335-55.

22. Lehner PJ, Davies KA, Walport MJ, et al. Meningococcal septicaemia in a C6-deficient patient and effects of plasma transfusion on lipopolysaccharide release. Lancet. Dec 5 1992;340(8832):1379-81. [Medline].

23. Leitao MF, Vilela MM, Rutz R, et al. Complement factor I deficiency in a family with recurrent infections. Immunopharmacology. Dec 1997;38(1-2):207-13. [Medline].

24. Lutz HU. How pre-existing, germline-derived antibodies and complement may help induce a primary immune response to nonself. Scand J Immunol. Mar 1999;49(3):224-8. [Medline].

25. Manzi S, Ahearn JM, Salmon J. New insights into complement: a mediator of injury and marker of disease activity in systemic lupus erythematosus. Lupus. 2004;13(5):298-303. [Medline].

26. Medof ME, Iida K, Mold C, Nussenzweig V. Unique role of the complement receptor CR1 in the degradation of C3b associated with immune complexes. J Exp Med. Dec 1 1982;156(6):1739-54. [Medline].

27. Pickering MC, Botto M, Taylor PR, et al. Systemic lupus erythematosus, complement deficiency, and apoptosis. Adv Immunol. 2000;76:227-324. [Medline].

28. Prodinger WM. Complement receptor type two (CR2,CR21): a target for influencing the humoral immune response and antigen-trapping. Immunol Res. 1999;20(3):187-94. [Medline].

29. Ross GD, Yount WJ, Walport MJ, et al. Disease-associated loss of erythrocyte complement receptors (CR1, C3b receptors) in patients with systemic lupus erythematosus and other diseases involving autoantibodies and/or complement activation. J Immunol. Sep 1985;135(3):2005-14. [Medline].

30. Rother K, Till GO, Hansch GM, eds. The Complement System. New York, NY: Springer-Verlag; 1997.

31. Schlesinger M, Nave Z, Levy Y, et al. Prevalence of hereditary properdin, C7 and C8 deficiencies in patients with meningococcal infections. Clin Exp Immunol. Sep 1990;81(3):423-7. [Medline].

32. Soderstrom C, Braconier JH, Danielsson D, Sjoholm AG. Bactericidal activity for Neisseria meningitidis in properdin-deficient sera. J Infect Dis. Jul 1987;156(1):107-12. [Medline].

33. Sullivan KE, Winkelstein JA. Genetically Determined Deficiencies of the Complement System. In: Ochs HD, Edvard Smith CI, Puck J. Primary Immunodeficiency Diseases: A Molecular and Genetic Approach. New York, NY: Oxford University Press; 1999:397-416.

34. Sumiya M, Super M, Tabona P, et al. Molecular basis of opsonic defect in immunodeficient children. Lancet. Jun 29 1991;337(8757):1569-70. [Medline].

35. Summerfield JA, Sumiya M, Levin M, Turner MW. Association of mutations in mannose binding protein gene with childhood infection in consecutive hospital series. BMJ. Apr 26 1997;314(7089):1229-32. [Medline].

36. Super M, Thiel S, Lu J, et al. Association of low levels of mannan-binding protein with a common defect of opsonisation. Lancet. Nov 25 1989;2(8674):1236-9. [Medline].

37. Tolnay M, Tsokos GC. Complement receptor 2 in the regulation of the immune response. Clin Immunol Immunopathol. Aug 1998;88(2):123-32. [Medline].

38. Turner MW. Mannose-binding lectin: the pluripotent molecule of the innate immune system. Immunol Today. Nov 1996;17(11):532-40. [Medline].

39. Volanakis JE, Frank MM, eds. The Human Complement System in Health and Disease. New York, NY: Marcel Dekker; 1998.

40. Walport MJ. Complement. First of two parts. N Engl J Med. Apr 5 2001;344(14):1058-66. [Medline].

41. Walport MJ. Complement. Second of two parts. N Engl J Med. Apr 12 2001;344(15):1140-4. [Medline].

42. Walport MJ, Davies KA, Botto M. C1q and systemic lupus erythematosus. Immunobiology. Aug 1998;199(2):265-85. [Medline].

43. Walport MJ, Lachmann PJ. Erythrocyte complement receptor type 1, immune complexes, and the rheumatic diseases. Arthritis Rheum. Feb 1988;31(2):153-8. [Medline].

44. Wisnieski JJ, Baer AN, Christensen J, et al. Hypocomplementemic urticarial vasculitis syndrome. Clinical and serologic findings in 18 patients. Medicine (Baltimore). Jan 1995;74(1):24-41. [Medline].

45. Zipfel PF, Heinen S, Jozsi M, Skerka C. Complement and diseases: defective alternative pathway control results in kidney and eye diseases. Mol Immunol. Jan 2006;43(1-2):97-106.

Further Reading

Keywords

C1qrs deficiency, C3 deficiency, C2-C4 deficiency, C5-9 deficiency, terminal membrane attack complex deficiency, MAC deficiency, mannan-binding lectin deficiency, MBL deficiency, immune system, bacterial infection, sepsis, host defense mechanism, immune complex disease, systemic lupus erythematosus, SLE, complement activation, disseminated meningococcal disease, Streptococcus pneumoniae, S pneumoniae, Haemophilus influenzae, H influenzae, Neisseria meningitidis, N meningitidis, meningococcemia

Contributor Information and Disclosures

Author

R Krishna Chaganti, MD, Assistant Clinical Professor, Division of Rheumatology, UCSF
R Krishna Chaganti, MD is a member of the following medical societies: American College of Rheumatology
Disclosure: Nothing to disclose.

Coauthor(s)

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

Stephen Rosenfeld, MD, Professor Emeritus, Department of Medicine, Allergy, Immunology and Rheumatology Unit, University of Rochester School of Medicine and Dentistry
Stephen Rosenfeld, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American Federation for Clinical Research, Clinical Immunology Society, and Medical Society of the State of New York
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Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
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Samuel R Marney, Jr, MD, Director, Associate Professor, Department of Internal Medicine, Division of Allergy and Immunology, Vanderbilt University School of Medicine
Samuel R Marney, Jr, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology, American College of Physicians, and Tennessee Medical Association
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CME Editor

Timothy D Rice, MD, Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, Saint Louis University School of Medicine
Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians
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Chief Editor

Michael A Kaliner, MD, Clinical Professor of Medicine, George Washington University School of Medicine; Chief, Section of Allergy and Immunology, Washington Hospital Center; Medical Director, Institute for Asthma and Allergy
Michael A Kaliner, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Allergy, Asthma and Immunology, American Society for Clinical Investigation, American Thoracic Society, and Association of American Physicians
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