Autosomal recessive hyperimmunoglobulin E syndrome (Q94304): Difference between revisions

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Síndrome de hiper IgE autossômica recessiva (AR-HIES) é um transtorno de imunodeficiência primária grave muito raro caracterizado pela tríade clínica de níveis séricos de IgE muito elevados, abscessos cutâneos estafilocócicos recorrentes e pneumonia recorrente. A tríade clínica é compartilhada com a síndrome HIES autossômica dominante (AD-HIES), que é mais frequente , mas outras manifestações, tais como infecções virais cutâneas persistentes, são exclusivas da AR-HIES.
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Autosomal recessive hyper IgE syndrome (AR-HIES) is a very rare severe primary immunodeficiency disorder characterised by the clinical triad of highly elevated serum IgE levels, recurring staphylococcal skin abscesses, and recurrent pneumonia. The clinical triad is shared with the more frequent autosomal dominant HIES syndrome (AD-HIES; ), but other features such as persistent cutaneous viral infections are unique to AR-HIES.

Revision as of 09:26, 16 August 2026

Autosomal recessive hyper IgE syndrome (AR-HIES) is a very rare severe primary immunodeficiency disorder characterised by the clinical triad of highly elevated serum IgE levels, recurring staphylococcal skin abscesses, and recurrent pneumonia. The clinical triad is shared with the more frequent autosomal dominant HIES syndrome (AD-HIES; ), but other features such as persistent cutaneous viral infections are unique to AR-HIES.
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ID_1909398175
    English
    Autosomal recessive hyperimmunoglobulin E syndrome
    Autosomal recessive hyper IgE syndrome (AR-HIES) is a very rare severe primary immunodeficiency disorder characterised by the clinical triad of highly elevated serum IgE levels, recurring staphylococcal skin abscesses, and recurrent pneumonia. The clinical triad is shared with the more frequent autosomal dominant HIES syndrome (AD-HIES; ), but other features such as persistent cutaneous viral infections are unique to AR-HIES.

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