| The hyper-IgE syndromes. | |
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MedLine Citation:
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PMID: 18424333 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The hyper IgE syndromes (HIES) are rare primary immune deficiencies characterized by elevated serum IgE, rash, and recurrent bacterial infections of the skin and lung. Autosomal dominant HIES, the most common disease in this group, results from STAT3 mutations and has a variety of connective tissue and skeletal abnormalities. The genetic etiologies of the more rare autosomal recessive forms still need delineation. Treatment of these syndromes has relied on prophylactic and therapeutic antimicrobial agents and aggressive skin care. The new and evolving genetic and immunologic understandings of this previously elusive set of diseases should lead to more effective disease-specific therapies. |
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Authors:
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Alexandra F Freeman; Steven M Holland |
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Publication Detail:
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Type: Journal Article; Research Support, N.I.H., Extramural; Review |
Journal Detail:
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Title: Immunology and allergy clinics of North America Volume: 28 ISSN: 0889-8561 ISO Abbreviation: Immunol Allergy Clin North Am Publication Date: 2008 May |
Date Detail:
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Created Date: 2008-04-21 Completed Date: 2008-08-12 Revised Date: 2012-03-07 |
Medline Journal Info:
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Nlm Unique ID: 8805635 Medline TA: Immunol Allergy Clin North Am Country: United States |
Other Details:
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Languages: eng Pagination: 277-91, viii Citation Subset: IM |
Affiliation:
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Laboratory of Clinical Infectious Disease, National Institute of Allergy and Infectious Disease, Building 10, CRC B3-4141, MSC 1684, 9000 Rockville Pike, Bethesda, MD 20892-1684, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Humans Immunoglobulin E / blood*, immunology Infection / etiology Job's Syndrome* / genetics, immunology, physiopathology, therapy STAT3 Transcription Factor / deficiency*, genetics, physiology |
| Grant Support | |
ID/Acronym/Agency:
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N01-CO12400/CO/NCI NIH HHS; Z01 AI000647-16/AI/NIAID NIH HHS; Z99 AI999999/AI/NIAID NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/STAT3 Transcription Factor; 37341-29-0/Immunoglobulin E |
| Comments/Corrections | |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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