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[Hereditary complement defects]

V Wahn

    Monatsschrift Kinderheilkunde : Organ Der Deutschen Gesellschaft Fur Kinderheilkunde
    |November 1, 1983
    PubMed

    Insights

    The complement system, a key human biological amplification system, can cause autoimmune disorders or infections when deficient. This review details complement component deficiencies, their clinical signs, and lab evaluation methods.

    Area of Science:

    • Immunology
    • Human Biology

    Context:

    • The complement system is a critical component of innate immunity.
    • Deficiencies in complement components are linked to significant health issues.

    Purpose:

    • To review the clinical manifestations and pathophysiology of known complement component deficiencies.
    • To outline laboratory evaluation procedures for suspected complement deficiency.

    Summary:

    • Hereditary deficiencies in complement components can result in autoimmune diseases, recurrent bacterial infections, or non-atopic cutaneous edema.
    • This review consolidates current knowledge on the clinical presentation and underlying mechanisms of these deficiencies.
    • Diagnostic laboratory evaluation protocols are presented for affected individuals.

    Impact:

    • Enhances understanding of complement system's role in health and disease.
    • Provides a clinical and diagnostic guide for healthcare professionals managing complement deficiencies.
    • Aids in the early identification and management of patients with recurrent infections or autoimmune conditions.

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