Complement Deficiencies (Pediatric)
What are complement deficiencies?
Complement proteins help to fight infections. In unusual circumstances, they are deficient.
What causes complement deficiencies?
There are many different types of complement proteins and they are usually inherited as autosomal recessive traits. Deficiencies may be associated with recurrent infections or with autoimmune disease like systemic lupus erythematosis and kidney problems. For example, C1q Deficiency is strongly linked to Pediatric Systemic Lupus Erythematosus. C5-C9 Deficiency is strongly associated with susceptibility to Neisseria species infections.
How are complement deficiencies diagnosed?
Complement deficiencies are diagnosed by evaluating complement classic and alternative pathways, levels and functions of individual complement components, and genetic testing.
What is the treatment for complement deficiencies?
Complement deficiencies that are associated with recurrent or severe infections are treated with prompt and aggressive antibiotic therapy for infections, frequent vaccinations, and, in severe cases, immunoglobulin treatment.