- Complement deficiency
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Complement deficiency Classification and external resources ICD-10 D84.1 ICD-9 279.8 OMIM 217000 120820, 120900, 610102 DiseasesDB 1847 1869, 1873, 7384, 34381 eMedicine med/419 ped/447 Complement deficiency is an immunodeficiency of absent or suboptimal functioning of one of the complement system proteins.[1]
The disorders can be divided into two categories:
- Disorders of the proteins that act to inhibit the complement system (such as C1-inhibitor) can lead to an overactive response, causing conditions such as hereditary angioedema and hemolytic-uremic syndrome.
- Disorders of the proteins that act to activate the complement system (such as C3) can lead to an underactive response, causing greater susceptibility to infections.
Because there are redundancies in the immune system, many complement disorders are never diagnosed. A recent study estimated that less than 10% are identified.[2]
Hypocomplementemia
The term "hypocomplementemia" is a more general term used to describe decreased complement levels.[3] The term "secondary complement disorder" is sometimes used to refer to low complement levels that are not directly due to a genetic cause but secondary to another medical condition.[4]
These levels can be used to distinguish conditions:
- Systemic lupus erythematosus causes low C3 and C4
- Membranoproliferative glomerulonephritis causes low C3, but normal C4
See also
References
- ^ Sherwood L. Gorbach; John G. Bartlett; Neil R. Blacklow (2004). Infectious diseases. Lippincott Williams & Wilkins. pp. 11–. ISBN 9780781733717. http://books.google.com/?id=91altE1evAsC&pg=PA11. Retrieved 30 May 2010.
- ^ Sjöholm AG, Jönsson G, Braconier JH, Sturfelt G, Truedsson L (2006). "Complement deficiency and disease: an update". Mol. Immunol. 43 (1–2): 78–85. doi:10.1016/j.molimm.2005.06.025. PMID 16026838.
- ^ "hypocomplementemia" at Dorland's Medical Dictionary
- ^ http://emedicine.medscape.com/article/136368-overview
Immune disorders: Lymphoid and complement immunodeficiency (D80–D85, 279.0–4) Primary IgA deficiency · IgG deficiency · IgM deficiency · Hyper IgM syndrome (2, 3, 4, 5) · Wiskott-Aldrich syndrome · Hyper-IgE syndromeOtherT cell deficiency (T)thymic hypoplasia: hypoparathyroid (Di George's syndrome) · euparathyroid (Nezelof syndrome, Ataxia telangiectasia)x-linked: X-SCID
autosomal: Adenosine deaminase deficiency · Omenn syndrome · ZAP70 deficiency · Bare lymphocyte syndromeAcquired Leukopenia:
LymphocytopeniaComplement deficiency C1-inhibitor (Angioedema/Hereditary angioedema) · Complement 2 deficiency/Complement 4 deficiency · MBL deficiency · Properdin deficiency · Complement 3 deficiency · Terminal complement pathway deficiency · Paroxysmal nocturnal hemoglobinuria · Complement receptor deficiencyCategories:- Disease stubs
- Noninfectious immunodeficiency-related cutaneous conditions
- Complement deficiency
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