Complement deficiency

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Complement deficiency
Classification and external resources
ICD-10D84.1
ICD-9279.8
OMIM217000 120820, 120900, 610102
DiseasesDB1847 1869, 1873, 7384, 34381
eMedicinemed/419 ped/447
 
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Complement deficiency
Classification and external resources
ICD-10D84.1
ICD-9279.8
OMIM217000 120820, 120900, 610102
DiseasesDB1847 1869, 1873, 7384, 34381
eMedicinemed/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:

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.

Individual complement levels can be used to distinguish conditions:

Vaccinations for encapsulated organisms is crucial for preventing infections in complement deficiencies.

See also[edit]

References[edit]

  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
  5. ^ Bruce L. Zuraw, M.D. (September 4, 2008). "Hereditary Angioedema". N Engl J Med 2008 359: 1027-1036. doi:10.1056/NEJMcp0803977 .