Complement deficiency

Complement deficiency
ICD-10	D84.1
ICD-9	279.8
OMIM	217000 120820, 120900, 610102
DiseasesDB	1847 Template:DiseasesDB2, Template:DiseasesDB2, Template:DiseasesDB2, Template:DiseasesDB2
eMedicine	med/419  ped/447

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Overview

Complement deficiency is a condition of absent or suboptimal functioning of one of the complement system proteins.

The disorders can be divided into two categories:

• Disorders of the proteins which 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 which 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.

See also

• Paroxysmal nocturnal hemoglobinuria

References

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