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Journal of Clinical Immunology
Published in: Journal of Clinical Immunology 2/2016

01-02-2016 | Letter to Editor

Autosomal Dominant Hyper IgE Syndrome – Treatment Strategies and Clinical Outcomes

Authors: Aisling M. Flinn, Andrew Cant, T. Ronan Leahy, Karina M. Butler, Andrew R. Gennery

Published in: Journal of Clinical Immunology | Issue 2/2016

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Excerpt

Autosomal dominant Hyper IgE Syndrome (AD-HIES) is a primary immunodeficiency characterized by connective tissue, vascular and skeletal abnormalities [1], and due to mutations in the signal transducer and activator of transcription 3 (STAT3) gene [2, 3]. Treatment is largely supportive and includes prophylactic antibiotics and antifungal therapy, early treatment of infections, immunoglobulin, and in some cases, haematopoietic stem cell transplant (HSCT) [4, 5]. There is little published evidence-based research on the management of AD-HIES in paediatric patients, particularly in preventing parenchymal lung damage. …
Literature
1.
2.
Holland SM, DeLeo FR, Elloumi HZ, Hsu AP, Uzel G, Brodsky N, et al. STAT3 mutations in the hyper-IgE syndrome. N Engl J Med. 2007;357(16):1608–19.CrossRefPubMed
3.
Minegishi Y, Saito M, Tsuchiya S. Dominant-negative mutations in the DNA-binding domain of STAT3 cause hyper-IgE syndrome. Nature. 2007;448:1058–62.CrossRefPubMed
4.
Patel NC, Gallagher JL, Torgerson TR, Gilman AL. Successful haploidentical donor hematopoietic stem cell transplant and restoration of STAT3 function in an adolescent with autosomal dominant hyper-IgE syndrome. J Clin Immunol. 2015;35(5):479–85.CrossRefPubMed
5.
Chandesris MO, Melki I, Natividad A, Puel A, Fieschi C, Yun L, et al. Autosomal dominant STAT3 deficiency and hyper-IgE syndrome: molecular, cellular, and clinical features from a French national survey. Medicine. 2012;91:e1–e19.PubMedCentralCrossRefPubMed
Metadata
Title
Autosomal Dominant Hyper IgE Syndrome – Treatment Strategies and Clinical Outcomes
Authors
Aisling M. Flinn
Andrew Cant
T. Ronan Leahy
Karina M. Butler
Andrew R. Gennery
Publication date
01-02-2016
Publisher
Springer US
Published in
Journal of Clinical Immunology / Issue 2/2016
Print ISSN: 0271-9142
Electronic ISSN: 1573-2592
DOI
https://doi.org/10.1007/s10875-015-0231-8

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