Frequency and clinical manifestations of patients with primary immunodeficiency disorders in Iran: update from the Iranian Primary Immunodeficiency Registry.
Primary immunodeficiency disorders (PID) are a heterogeneous group of diseases, characterized by an increased susceptibility to infections. A total of 930 patients (573 males and 357 females) are registered in Iranian PID Registry (IPIDR) during three decades. Predominantly antibody deficiencies were the most common (38.4%), followed by congenital defects of phagocyte number and/or function (28.3%), other well-defined immunodeficiency syndromes (17.7%), combined T- and B-cell immunodeficiencies (11.0%), complement deficiencies (2.4%), and diseases of immune dysregulation (2.3%). Common variable immunodeficiency was the most frequent disorder (20.8%), followed by chronic granulomatous disease, ataxia-telangiectasia, btk deficiency, selective IgA deficiency, and T-B-severe combined immunodeficiency. The frequency of other PID disorders was less than 50 in number (<5%). There is an increasing trend in recognition of more PID in the recent years. Construction of such registry is not only important for its epidemiological aspect but also for its role in increasing the physician's knowledge about such disorders.
Rezaei
Nima
N
Department of Allergy and Clinical Immunology of Children Medical Center, Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran. rezaei_nima@hbi.ir
Aghamohammadi
Asghar
A
Moin
Mostafa
M
Pourpak
Zahra
Z
Movahedi
Masoud
M
Gharagozlou
Mohammad
M
Atarod
Lida
L
Ghazi
Bahram Mirsaeid
BM
Isaeian
Anna
A
Mahmoudi
Maryam
M
Abolmaali
Kamran
K
Mansouri
Davoud
D
Arshi
Saba
S
Tarash
Naser Javaher
NJ
Sherkat
Roya
R
Akbari
Hedayat
H
Amin
Reza
R
Alborzi
Abdolvahab
A
Kashef
Sara
S
Farid
Reza
R
Mohammadzadeh
Iraj
I
Shabestari
Mehrnaz Sadeghi
MS
Nabavi
Mohammad
M
Farhoudi
Abolhassan
A
D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D003241:Consanguinity; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000453:epidemiology* / Q000235:genetics / Q000401:mortality; D007223:Infant; D007231:Infant, Newborn; D007492:Iran / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D012042:Registries