Complement deficiency

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Complement deficiency
ICD-10 D84.1
ICD-9 279.8
OMIM 217000 120820, 120900, 610102
DiseasesDB 1847 1869, 1873, 7384, 34381

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and Keywords: Hypocomplementemia

Overview

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.

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:

See also

References

  1. Sherwood L. Gorbach; John G. Bartlett; Neil R. Blacklow (2004). Infectious diseases. Lippincott Williams & Wilkins. pp. 11–. ISBN 978-0-7817-3371-7. Retrieved 30 May 2010.
  2. 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.
  3. hypocomplementemia at Dorland's Medical Dictionary
  4. http://emedicine.medscape.com/article/136368-overview

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