Dominant-negative signal transducer and activator of transcription (STAT)3 variants in adult patients: A single center experience.
adults
dupilumab
hyper IgE syndrome
inborn errors of imunity
stat3
Journal
Frontiers in immunology
ISSN: 1664-3224
Titre abrégé: Front Immunol
Pays: Switzerland
ID NLM: 101560960
Informations de publication
Date de publication:
2022
2022
Historique:
received:
15
09
2022
accepted:
07
12
2022
entrez:
6
1
2023
pubmed:
7
1
2023
medline:
10
1
2023
Statut:
epublish
Résumé
Autosomal dominant hyper-IgE syndrome (AD-HIES) caused by dominant negative (DN) variants in the signal transducer and activator of transcription 3 gene ( To describe our experience with the diagnosis and treatment of adult patients with AD-HIES induced by DN- The medical records of adult patients (>18 years) treated at the Allergy and Clinical Immunology Clinic of Hadassah Medical Center, Jerusalem, Israel, were retrospectively analyzed. Immune and genetic workups were used to confirm diagnosis. Three adult patients (2 males; age 29-41 years) were diagnosed with DN- Dupilumab can be used safely as a biotherapy for atopic dermatitis in these patients as it can effectively alleviate eczema-related symptoms. Immunologists and dermatologists treating AD-HIES adult patients should be aware of demodicosis as a possible manifestation. DN-
Sections du résumé
Background
Autosomal dominant hyper-IgE syndrome (AD-HIES) caused by dominant negative (DN) variants in the signal transducer and activator of transcription 3 gene (
Objectives
To describe our experience with the diagnosis and treatment of adult patients with AD-HIES induced by DN-
Methods
The medical records of adult patients (>18 years) treated at the Allergy and Clinical Immunology Clinic of Hadassah Medical Center, Jerusalem, Israel, were retrospectively analyzed. Immune and genetic workups were used to confirm diagnosis.
Results
Three adult patients (2 males; age 29-41 years) were diagnosed with DN-
Discussion
Dupilumab can be used safely as a biotherapy for atopic dermatitis in these patients as it can effectively alleviate eczema-related symptoms. Immunologists and dermatologists treating AD-HIES adult patients should be aware of demodicosis as a possible manifestation. DN-
Identifiants
pubmed: 36605204
doi: 10.3389/fimmu.2022.1044933
pmc: PMC9807906
doi:
Substances chimiques
STAT3 Transcription Factor
0
STAT3 protein, human
0
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
1044933Informations de copyright
Copyright © 2022 Shamriz, Rubin, Simon, Lev, Barel, Somech, Korem, Matza Porges, Freund, Hagin, Garty, Nahum, Molho Pessach and Tal.
Déclaration de conflit d'intérêts
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
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