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[Hereditary angioedema].

E W Nielsen, H Skjeflo

    Den Norske Tannlaegeforenings Tidende
    |January 1, 1991
    PubMed
    Summary
    This summary is machine-generated.

    Hereditary angioedema (HAE) is a rare genetic disorder causing unpredictable swelling. Treatment involves C1 inhibitor concentrate for acute attacks and prophylactic medication for frequent episodes.

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    Area of Science:

    • Immunology
    • Genetics

    Context:

    • Hereditary angioedema (HAE) is a rare autosomal dominant disorder.
    • Characterized by recurrent episodes of subcutaneous and submucosal swelling.
    • Can lead to life-threatening laryngeal edema.

    Purpose:

    • To describe the pathophysiology and clinical manifestations of HAE.
    • To outline current management strategies for HAE.
    • To differentiate HAE from allergic reactions.

    Summary:

    • HAE results from a deficiency in active C1 inhibitor.
    • Edema is not allergic; antihistamines are ineffective.
    • Prophylactic medication is recommended before procedures like surgery or dental extractions.
    • Acute attacks are treated with C1 inhibitor concentrate.

    Impact:

    • Improved understanding of HAE pathophysiology.
    • Guidance on appropriate treatment and prophylaxis.
    • Enhanced patient management and outcomes for HAE.