title: Consanguinity in primary immunodeficiency disorders; the report from Iranian Primary Immunodeficiency Registry.
abstract: OBJECTIVE
  Primary Immunodeficiency Disorders (PiD) are a heterogeneous group of genetic disorders, with different modes of inheritance. This study was accomplished in order to determine the frequency of consanguineous marriages in the families of patients with PiD.
  
  
  METHODS
  In this study, the records 515 Iranian PiD patients were reviewed during a 25-year period.
  
  
  RESULTS
  The mean proportion of consanguineous marriages was 65.6% among PiD patients, while the overall rate was 38.6% in the country. The rate of consanguinity was 77.8% in cellular immunodeficiencies, 75.8% in combined immunodeficiencies, 72.5% in defects of phagocytic function, 58.6% in other immunodefiiencies, 54.1% in predominantly antibody deficiencies, and 50% in complement deficiencies. Moreover all patients with immunodeficiency associated with other diseases had consanguineous parents. Such marriages were most common in the parents of patients with Chediak-Higashi syndrome, severe combined immunodeficiencies, primary CD4 deficiency, ataxia-telangiectasia, selective IgG class deficiencies, chronic granulomatous disease, and Schwachman syndrome.
  
  
  CONCLUSIONS
  It is important to inform the general population about the dangers of consanguinity, which is very common in some areas such as Iran. Premarital examination to avoid genetic diseases could be suggested, especially in a community where the rate of consanguineous marriage is high.
authors.affiliation: Department of Allergy and Clinical Immunology, Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Iran. rezaei_nima@hbi.ir
authors.forename: Nima
authors.initials: N
authors.lastname: Rezaei
authors.forename: Zahra
authors.initials: Z
authors.lastname: Pourpak
authors.forename: Asghar
authors.initials: A
authors.lastname: Aghamohammadi
authors.forename: Abolhassan
authors.initials: A
authors.lastname: Farhoudi
authors.forename: Masoud
authors.initials: M
authors.lastname: Movahedi
authors.forename: Mohammad
authors.initials: M
authors.lastname: Gharagozlou
authors.forename: Bahram
authors.initials: B
authors.lastname: Mirsaeid Ghazi
authors.forename: Lida
authors.initials: L
authors.lastname: Atarod
authors.forename: Kamran
authors.initials: K
authors.lastname: Abolmaali
authors.forename: Maryam
authors.initials: M
authors.lastname: Mahmoudi
authors.forename: Davoud
authors.initials: D
authors.lastname: Mansouri
authors.forename: Saba
authors.initials: S
authors.lastname: Arshi
authors.forename: Naser Javaher
authors.initials: NJ
authors.lastname: Tarash
authors.forename: Roya
authors.initials: R
authors.lastname: Sherkat
authors.forename: Reza
authors.initials: R
authors.lastname: Amin
authors.forename: Sara
authors.initials: S
authors.lastname: Kashef
authors.forename: Reza Farid
authors.initials: RF
authors.lastname: Hosseini
authors.forename: Iraj
authors.initials: I
authors.lastname: Mohammadzadeh
authors.forename: Mehrnaz Sadeghi
authors.initials: MS
authors.lastname: Shabestari
authors.forename: Mohammad
authors.initials: M
authors.lastname: Nabavi
authors.forename: Mostafa
authors.initials: M
authors.lastname: Moin
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D003241:Consanguinity; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000453:epidemiology*; D007492:Iran / Q000453:epidemiology; D008297:Male; D010375:Pedigree; D012042:Registries