Complement deficiency

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Complement deficiency
Classification and external resources
Specialty hematology
ICD-10 D84.1
ICD-9-CM 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 activate the complement system (such as C3) can lead to an underactive response, causing greater susceptibility to infections.[citation needed]

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[edit]

Hypocomplementemia may be used more generally to refer to decreased complement levels[3] while secondary complement disorder is to low complement levels that are not directly due to a genetic cause but secondary to another medical condition.[4]

The total hemolytic complement complement CH50 level in the blood will be low or undetectable with complement deficiencies.[citation needed]

Individual complement levels can be used to distinguish conditions:

Vaccinations for encapsulated organisms is crucial for preventing infections in complement deficiencies.[citation needed]

Epidemiology[edit]

C2 deficiency is prevalent in 120,000 people in Western countries.[7] It occurs in about 1 in 10,000 persons.[8]

See also[edit]

References[edit]

  1. ^ Winkelstein, Jerry A. (2004). "The Complement System". In Gorbach, Sherwood L.; Bartlett, John G.; Blacklow, Neil R. Infectious Diseases. Lippincott Williams & Wilkins. pp. 8–13. ISBN 978-0-7817-3371-7. 
  2. ^ Sjöholm, A.G.; Jönsson, G.; Braconier, J.H.; Sturfelt, G.; Truedsson, L. (2006). "Complement deficiency and disease: An update". Molecular Immunology 43 (1–2): 78–85. doi:10.1016/j.molimm.2005.06.025. PMID 16026838. 
  3. ^ "hypocomplementemia" at Dorland's Medical Dictionary
  4. ^ Complement-Related Disorders at eMedicine
  5. ^ Fauci, Anthony; Braunwald, Eugene; Kasper, Dennis; Hauser, Stephen; Longo, Dan; Jameson, J.; Loscalzo, Joseph, eds. (2008). Harrison's Principles of Internal Medicine (17th ed.). ISBN 978-0-07-146633-2. [page needed]
  6. ^ Zuraw, Bruce L. (2008). "Hereditary Angioedema". New England Journal of Medicine 359 (10): 1027–36. doi:10.1056/NEJMcp0803977. PMID 18768946. 
  7. ^ http://www.patient.co.uk/doctor/complement-deficiencies[full citation needed]
  8. ^ http://www.omim.org/entry/217000[full citation needed]