Autosomal recessive hyperimmunoglobulin E syndrome (Q94304): Difference between revisions
From determinar.ia.br - Determine suas informações
Created a new Item |
Changed an Item |
||||||||||||||
| (6 intermediate revisions by the same user not shown) | |||||||||||||||
| description / pt-br | description / pt-br | ||||||||||||||
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. | |||||||||||||||
| description / en | description / en | ||||||||||||||
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. | |||||||||||||||
| Property / Canonical URI | |||||||||||||||
| Property / Canonical URI: https://id.who.int/icd/entity/1909398175 / rank | |||||||||||||||
Normal rank | |||||||||||||||
| Property / CURIE | |||||||||||||||
CID11:ID_1909398175 | |||||||||||||||
| Property / CURIE: CID11:ID_1909398175 / rank | |||||||||||||||
Normal rank | |||||||||||||||
| Property / Canary Token | |||||||||||||||
dki-india-ID_1909398175 | |||||||||||||||
| Property / Canary Token: dki-india-ID_1909398175 / rank | |||||||||||||||
Normal rank | |||||||||||||||
| Property / Verification Status | |||||||||||||||
Concluído | |||||||||||||||
| Property / Verification Status: Concluído / rank | |||||||||||||||
Normal rank | |||||||||||||||
| Property / Knowledge Architect | |||||||||||||||
| Property / Knowledge Architect: https://pauloleads.com.br/cases-publicos/ / rank | |||||||||||||||
Normal rank | |||||||||||||||
| Property / Collection date | |||||||||||||||
15 August 2026
| |||||||||||||||
| Property / Collection date: 15 August 2026 / rank | |||||||||||||||
Normal rank | |||||||||||||||
Latest 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.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | 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. |
Statements
CID11:ID_1909398175
0 references
dki-india-ID_1909398175
0 references
Concluído
0 references
15 August 2026
0 references
