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Phenotyping and long-term follow up of patients with hyper IgE syndrome
Alyasin, S; Esmaeilzadeh, H; Ebrahimi, N; Nabavizadeh, SH; Kashef, S; Esmaeilzadeh, E; Babaei, M; Amin, R.
Afiliação
  • Alyasin, S; Shiraz University of Medical Sciences. Allegry Research Center. Shiraz. Uran
  • Esmaeilzadeh, H; Shiraz University of Medical Sciences. Allegry Research Center. Shiraz. Uran
  • Ebrahimi, N; Shiraz University of Medical Sciences. Allegry Research Center. Shiraz. Uran
  • Nabavizadeh, SH; Shiraz University of Medical Sciences. Allegry Research Center. Shiraz. Uran
  • Kashef, S; Shiraz University of Medical Sciences. Namazi Hospital. Department of Allergy and Clinical Immunology. Shiraz. Iran
  • Esmaeilzadeh, E; Shiraz University of Medical Sciences. Division of Rheumatology. Department of Internal Medicine. Shiraz. Iran
  • Babaei, M; Shiraz University of Medical Sciences. Allegry Research Center. Shiraz. Uran
  • Amin, R; Shiraz University of Medical Sciences. Allegry Research Center. Shiraz. Uran
Allergol. immunopatol ; 47(2): 152-158, mar.-abr. 2019. graf, tab
Artigo em Espanhol | IBECS | ID: ibc-180803
Biblioteca responsável: ES1.1
Localização: BNCS
ABSTRACT
Introduction and

objectives:

Long-term follow up of patients with hyper IgE syndrome (HIES), as a primary immunodeficiency disorder, has been poorly investigated. This study describes common clinical and immunological features of patients with HIES in the last 10 years in Shiraz University of Medical Sciences, Shiraz, Iran. Methods and patients In this cross-sectional study, the symptoms and medical records of 18 patients, who were diagnosed with HIES, were observed. Genetic and immunologic study was also performed.

Results:

Eighteen patients with the mean age of 13 years old were investigated. Ten patients were detected to have mutations in DOCK8 gene and autosomal recessive HIES (AR-HIES); and four patients were found with STAT3 mutation and autosomal dominant HIES (AD-HIES). So, 14 patients with known genetic results were considered for further data analysis. Food allergy, eczema, viral and skin infections were the major complications of AR-HIES patients. The major clinical complications of AD-HIES patients were pneumonia, skin infections and eczema. Food allergy and viral infection were significantly higher in DOCK8 deficient patients. The most common causes of hospitalization in both AR-HIES and AD-HIES patients were pneumonia, skin infections and sepsis. The most common cause of death was found to be sepsis. Conclusions; AD-HIES and AR-HIES cannot be differentiated only based on the clinical presentations. Genetic features are also necessary for better diagnosis. This study, summarizing the clinical, immunological and genetic information of the patients with AD-HIES and AR-HIES, may open a way for better diagnosis and management of HIES
RESUMEN
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Assuntos
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Coleções: Bases de dados nacionais / Espanha Base de dados: IBECS Assunto principal: Pneumonia / Hipersensibilidade Alimentar / Síndrome de Job / Mutação Limite: Adolescente / Adulto / Criança / Criança, pré-escolar / Humanos / Masculino Idioma: Espanhol Revista: Allergol. immunopatol Ano de publicação: 2019 Tipo de documento: Artigo Instituição/País de afiliação: Shiraz University of Medical Sciences/Iran / Shiraz University of Medical Sciences/Uran
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Coleções: Bases de dados nacionais / Espanha Base de dados: IBECS Assunto principal: Pneumonia / Hipersensibilidade Alimentar / Síndrome de Job / Mutação Limite: Adolescente / Adulto / Criança / Criança, pré-escolar / Humanos / Masculino Idioma: Espanhol Revista: Allergol. immunopatol Ano de publicação: 2019 Tipo de documento: Artigo Instituição/País de afiliação: Shiraz University of Medical Sciences/Iran / Shiraz University of Medical Sciences/Uran
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