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Phenotyping and follow up of forty-seven Iranian patients with common variable immunodeficiency
Arshi, Saba; Nabavi, Mohammad; Hasan Bemanian, Mohammad; Shakeri, Ramin; Taghvaei, Behrang; Ghalebaghi, Babak; Babaie, Delara; Bahrami, Ahmad; Fallahpour, Morteza; Esmaeilzadeh, Hossein; Rekabi, Mahsa; Amadian, Javad; Eslami, Narjes; Shokri, Sima; Jalali, Farhad; Akbarpour, Nadieh; Molatefi, Rasol; Rezaei, Nima.
Afiliação
  • Arshi, Saba; Iran University of Medical Sciences (IUMS). Allergy and Clinical Immunology Department of Rasol-E-Akram Hospital. Tehran. Iran
  • Nabavi, Mohammad; Iran University of Medical Sciences (IUMS). Allergy and Clinical Immunology Department of Rasol-E-Akram Hospital. Tehran. Iran
  • Hasan Bemanian, Mohammad; Iran University of Medical Sciences (IUMS). Allergy and Clinical Immunology Department of Rasol-E-Akram Hospital. Tehran. Iran
  • Shakeri, Ramin; Tehran University of Medical Sciences (TUMS). Digestive Disease Research Institute. Digestive Oncology Research Center. Tehran. Irán
  • Taghvaei, Behrang; Iran University of Medical Sciences (IUMS). Allergy and Clinical Immunology Department of Rasol-E-Akram Hospital. Tehran. Iran
  • Ghalebaghi, Babak; Iran University of Medical Sciences (IUMS). Allergy and Clinical Immunology Department of Rasol-E-Akram Hospital. Tehran. Iran
  • Babaie, Delara; Shaheed Beheshti University of Medical Sciences (SBMU). Allergy and Clinical Immunology Department of Mofid Hospital. Tehran. Iran
  • Bahrami, Ahmad; Iran University of Medical Sciences (IUMS). Allergy and Clinical Immunology Department of Rasol-E-Akram Hospital. Tehran. Iran
  • Fallahpour, Morteza; Iran University of Medical Sciences (IUMS). Allergy and Clinical Immunology Department of Rasol-E-Akram Hospital. Tehran. Iran
  • Esmaeilzadeh, Hossein; Shiraz University of Medical Sciences. Allergy Research Center. Shiraz. Iran
  • Rekabi, Mahsa; Iran University of Medical Sciences (IUMS). Allergy and Clinical Immunology Department of Rasol-E-Akram Hospital. Tehran. Iran
  • Amadian, Javad; Iran University of Medical Sciences (IUMS). Allergy and Clinical Immunology Department of Rasol-E-Akram Hospital. Tehran. Iran
  • Eslami, Narjes; Iran University of Medical Sciences (IUMS). Allergy and Clinical Immunology Department of Rasol-E-Akram Hospital. Tehran. Iran
  • Shokri, Sima; Iran University of Medical Sciences (IUMS). Allergy and Clinical Immunology Department of Rasol-E-Akram Hospital. Tehran. Iran
  • Jalali, Farhad; Iran University of Medical Sciences (IUMS). Allergy and Clinical Immunology Department of Rasol-E-Akram Hospital. Tehran. Iran
  • Akbarpour, Nadieh; Iran University of Medical Sciences (IUMS). Allergy and Clinical Immunology Department of Rasol-E-Akram Hospital. Tehran. Iran
  • Molatefi, Rasol; Iran University of Medical Sciences (IUMS). Allergy and Clinical Immunology Department of Rasol-E-Akram Hospital. Tehran. Iran
  • Rezaei, Nima; Tehran University of Medical Sciences (TUMS). School of Medicine. Molecular Immunology Research Center and Department of Immunology. Tehran. Iran
Allergol. immunopatol ; 44(3): 226-231, mayo-jun. 2016. tab
Artigo em Inglês | IBECS | ID: ibc-152078
Biblioteca responsável: ES1.1
Localização: BNCS
ABSTRACT

BACKGROUND:

Common variable immune deficiency (CVID) is a heterogeneous syndrome with a wide variety of signs and symptoms. This study describes the phenotyping and survival of the CVID patients in the allergy and clinical immunology department of Rasol-E-Akram Hospital of Iran University of Medical Sciences in Tehran.

METHOD:

We retrospectively reviewed hospital files of CVID patients in our department until January 2014. All patients were diagnosed with standard diagnostic criteria of CVID, treated and visited monthly, during the follow-up period. We divided the patients into four phenotypes; infection only, cytopenia, polyclonal lymphocytic infiltration and unexplained enteropathy. The immunologic, demographic and clinical findings in different phenotypes were analysed.

RESULTS:

The study included 47 CVID patients with mean age at onset of symptoms and diagnosis of 11.2 and 20.2 years, respectively. Phenotyping of our patients was only infection (62%), cytopenia (26%) and PLI (19%) and 94% of cases had only one phenotype. We did not find a significant relation between the clinical phenotypes and immunologic or demographic data. Rate of parental consanguinity in our cases was 47%. Parental consanguinity was related to lower age at onset, lower age at diagnosis and higher baseline IgG levels. Patients with malignancy and autoimmunity had significantly higher age at onset. Our patients were followed-up for 6.9 years and the mortality rate during this time was 6%.

CONCLUSIONS:

Parental consanguinity and age at onset of CVID symptoms may have important roles in CVID manifestations
RESUMEN
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Assuntos
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Coleções: Bases de dados nacionais / Espanha Base de dados: IBECS Assunto principal: Fenótipo / Autoimunidade / Morbidade / Mortalidade / Imunodeficiência de Variável Comum / Consanguinidade / 50293 Tipo de estudo: Estudo observacional / Estudo prognóstico / Fatores de risco Limite: Feminino / Humanos / Masculino País/Região como assunto: Ásia Idioma: Inglês Revista: Allergol. immunopatol Ano de publicação: 2016 Tipo de documento: Artigo Instituição/País de afiliação: Iran University of Medical Sciences (IUMS)/Iran / Shaheed Beheshti University of Medical Sciences (SBMU)/Iran / Shiraz University of Medical Sciences/Iran / Tehran University of Medical Sciences (TUMS)/Iran / Tehran University of Medical Sciences (TUMS)/Irán
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Coleções: Bases de dados nacionais / Espanha Base de dados: IBECS Assunto principal: Fenótipo / Autoimunidade / Morbidade / Mortalidade / Imunodeficiência de Variável Comum / Consanguinidade / 50293 Tipo de estudo: Estudo observacional / Estudo prognóstico / Fatores de risco Limite: Feminino / Humanos / Masculino País/Região como assunto: Ásia Idioma: Inglês Revista: Allergol. immunopatol Ano de publicação: 2016 Tipo de documento: Artigo Instituição/País de afiliação: Iran University of Medical Sciences (IUMS)/Iran / Shaheed Beheshti University of Medical Sciences (SBMU)/Iran / Shiraz University of Medical Sciences/Iran / Tehran University of Medical Sciences (TUMS)/Iran / Tehran University of Medical Sciences (TUMS)/Irán
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