Document Detail


Hyper-IgE syndrome with STAT3 mutation: a case report in Mainland China.
MedLine Citation:
PMID:  20490271     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Hyper-immunoglobulin E syndromes (HIES) including compound primary immunodeficiency and nonimmunological abnormalities are characterized by extremely high serum IgE levels, eosinophilia, eczema, susceptibility to infections, distinctive facial appearance, retention of deciduous teeth, cyst-forming pneumonias, and skeletal abnormalities. Itis reported that some cases of familial HIES are relative to autosomal dominant or recessive inheritance, but most cases are sporadic, and result from mutations in the human signal transducer and activator of transcription 3 (STAT3) gene. In this paper, we firstly report a young man diagnosed of Hyper-IgE syndrome with STAT3 mutation in Mainland China, and investigate the autosomal dominant trait of his family members.
Authors:
Lixin Xie; Xiaoxiang Hu; Yang Li; Weihua Zhang; Liang'an Chen
Publication Detail:
Type:  Case Reports; Journal Article     Date:  2010-05-17
Journal Detail:
Title:  Clinical & developmental immunology     Volume:  2010     ISSN:  1740-2530     ISO Abbreviation:  Clin. Dev. Immunol.     Publication Date:  2010  
Date Detail:
Created Date:  2010-05-21     Completed Date:  2010-09-14     Revised Date:  2010-09-28    
Medline Journal Info:
Nlm Unique ID:  101183692     Medline TA:  Clin Dev Immunol     Country:  England    
Other Details:
Languages:  eng     Pagination:  289873     Citation Subset:  IM    
Affiliation:
Department of Respiratory Medicine, Chinese PLA General Hospital, 28th Fuxing Road, Beijing 100853, China.
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MeSH Terms
Descriptor/Qualifier:
China
DNA Mutational Analysis
Eczema
Humans
Immunoglobulin E / blood
Job's Syndrome / blood,  complications,  diagnosis,  genetics*,  physiopathology
Lung / abnormalities,  microbiology,  radiography,  surgery
Male
Mutation / genetics*
Pedigree
Pneumonia, Staphylococcal / blood,  diagnosis,  etiology,  genetics*,  physiopathology
STAT3 Transcription Factor / biosynthesis,  genetics*
Staphylococcus aureus / immunology*,  pathogenicity
Tomography, X-Ray Computed
Young Adult
Chemical
Reg. No./Substance:
0/STAT3 Transcription Factor; 37341-29-0/Immunoglobulin E
Comments/Corrections

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