title: Long-term evaluation of a historical cohort of Iranian common variable immunodeficiency patients.
abstract: OBJECTIVE
  Common variable immune deficiency (CVID) is the most frequent form of symptomatic primary immunodeficiency disease, characterized by hypogammaglobulinemia, recurrent infections and increased predisposition to autoimmunity and malignancies. The aim of this study was to reconsider important points of previously performed studies on Iranian CVID patients diagnosed and followed from 1984 to 2013.
  
  
  METHODS
  Diagnosis was made using approved criteria including reductions of serum levels of immunoglobulins and exclusion of well-known single gene defects in individuals with an age >4 years and evidence of specific antibody deficiency.
  
  
  RESULTS
  Detailed information on demographic data, survival rates, clinical phenotypes, immunologic and genetic data and treatment of 173 patients are provided. The early onset presentation (74.5%) and rate of consanguineous marriage (61.2%) were considerably higher in our cohort. Our study revealed clinically related correlations regarding consanguinity, the population of naïve CD4(+) T cells and switched-memory B cells, cytokine levels and special genetic factors (including HLA and AID genes).
  
  
  CONCLUSIONS
  Despite current efforts, more comprehensive studies are needed, especially for classification and investigation of the genetic background and prognostic factors for patients with CVID in order to better managment and followup of patinets.
authors.affiliation: Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Science, 62 Qarib St., Keshavarz Blvd., Tehran 14194, Iran.
authors.forename: Asghar
authors.initials: A
authors.lastname: Aghamohammadi
authors.forename: Hassan
authors.initials: H
authors.lastname: Abolhassani
authors.forename: AmirHossein
authors.initials: A
authors.lastname: Latif
authors.forename: Firouzeh
authors.initials: F
authors.lastname: Tabassomi
authors.forename: Tahaamin
authors.initials: T
authors.lastname: Shokuhfar
authors.forename: Babak
authors.initials: B
authors.lastname: Torabi Sagvand
authors.forename: Shervin
authors.initials: S
authors.lastname: Shahinpour
authors.forename: Babak
authors.initials: B
authors.lastname: Mirminachi
authors.forename: Nima
authors.initials: N
authors.lastname: Parvaneh
authors.forename: Masoud
authors.initials: M
authors.lastname: Movahedi
authors.forename: Mohammad
authors.initials: M
authors.lastname: Gharagozlou
authors.forename: Roya
authors.initials: R
authors.lastname: Sherkat
authors.forename: Reza
authors.initials: R
authors.lastname: Amin
authors.forename: Soheila
authors.initials: S
authors.lastname: Aleyasin
authors.forename: Reza
authors.initials: R
authors.lastname: Faridhosseini
authors.forename: Farahzad
authors.initials: F
authors.lastname: Jabbari-Azad
authors.forename: Taher
authors.initials: T
authors.lastname: Cheraghi
authors.forename: Mohamad Hosein
authors.initials: MH
authors.lastname: Eslamian
authors.forename: Abbas
authors.initials: A
authors.lastname: Khalili
authors.forename: Najmoddin
authors.initials: N
authors.lastname: Kalantari
authors.forename: Alireza
authors.initials: A
authors.lastname: Shafiei
authors.forename: Abbas
authors.initials: A
authors.lastname: Dabbaghzade
authors.forename: Alireza
authors.initials: A
authors.lastname: Khayatzadeh
authors.forename: Mohsen
authors.initials: M
authors.lastname: Ebrahimi
authors.forename: Davood
authors.initials: D
authors.lastname: Razavinejad
authors.forename: Saeid
authors.initials: S
authors.lastname: Bazregari
authors.forename: Mehran
authors.initials: M
authors.lastname: Ebrahimi
authors.forename: Javad
authors.initials: J
authors.lastname: Ghaffari
authors.forename: Mohammad Hassan
authors.initials: MH
authors.lastname: Bemanian
authors.forename: Nasrin
authors.initials: N
authors.lastname: Behniafard
authors.forename: Sara
authors.initials: S
authors.lastname: Kashef
authors.forename: Iraj
authors.initials: I
authors.lastname: Mohammadzadeh
authors.forename: Lennart
authors.initials: L
authors.lastname: Hammarström
authors.forename: Nima
authors.initials: N
authors.lastname: Rezaei
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D017074:Common Variable Immunodeficiency / Q000453:epidemiology / Q000235:genetics / Q000276:immunology; D005260:Female; D006801:Humans; D007492:Iran / Q000453:epidemiology; D008297:Male; D055815:Young Adult
keywords: clinical manifestation; common variable immunodeficiency; epidemiology; laboratory characteristic; retrospective cohort; treatment