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Complement Deficiency, Pediatric

Melanie M. Makhija, MD, MSc Reviewed 10/2018
 


BASICS

DESCRIPTION

  • Complement is a major component of the innate immune system.

    • Consists of plasma and membrane proteins which mediate three pathways of cascading enzyme reactions (classical, alternativ...

DIAGNOSIS

HISTORY

Indications for evaluating complement system: 
  • Recurrent pyogenic infections in patients with normal white blood cell count and immunoglobulin levels

  • Recurrent neisserial infections (me...

TREATMENT

GENERAL MEASURES

  • Consider prophylactic antibiotics to prevent recurrent infections.

  • Vaccination with Streptococcus pneumoniae and Neisseria meningitidis conjugate vaccines as well as Haemophil...

ONGOING CARE

COMPLICATIONS

  • Severe infections and sequelae including death

  • Immune complex disease

  • Autoimmunity

ADDITIONAL READING

  • Bonilla FA, Geha RS. 12. Primary immunodeficiency diseases. J Allergy Clin Immunol.  2003;111(Suppl 2):S571–S581. [View Abstract on OvidInsights]

  • Frank MM. Complement deficienci...

CODES

ICD9

279.8 Other specified disorders involving the immune mechanism 

ICD10

D84.1 Defects in the complement system 

SNOMED

  • 234593008 Classical complement pathway abnormality (disorder)

  • 234594002 Comple...

FAQ

  • Q: When should I evaluate for a complement deficiency?

  • A: Any child with recurrent sinopulmonary infections/invasive pneumococcal infections or >1 episode of a neisserial infection.

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