Consanguinity in primary immunodeficiency disorders; the report from Iranian Primary Immunodeficiency Registry.
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.
Rezaei
Nima
N
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
Pourpak
Zahra
Z
Aghamohammadi
Asghar
A
Farhoudi
Abolhassan
A
Movahedi
Masoud
M
Gharagozlou
Mohammad
M
Mirsaeid Ghazi
Bahram
B
Atarod
Lida
L
Abolmaali
Kamran
K
Mahmoudi
Maryam
M
Mansouri
Davoud
D
Arshi
Saba
S
Tarash
Naser Javaher
NJ
Sherkat
Roya
R
Amin
Reza
R
Kashef
Sara
S
Hosseini
Reza Farid
RF
Mohammadzadeh
Iraj
I
Shabestari
Mehrnaz Sadeghi
MS
Nabavi
Mohammad
M
Moin
Mostafa
M
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