title: Frequency and clinical manifestations of patients with primary immunodeficiency disorders in Iran: update from the Iranian Primary Immunodeficiency Registry.
abstract: 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.
authors.affiliation: 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
authors.forename: Nima
authors.initials: N
authors.lastname: Rezaei
authors.forename: Asghar
authors.initials: A
authors.lastname: Aghamohammadi
authors.forename: Mostafa
authors.initials: M
authors.lastname: Moin
authors.forename: Zahra
authors.initials: Z
authors.lastname: Pourpak
authors.forename: Masoud
authors.initials: M
authors.lastname: Movahedi
authors.forename: Mohammad
authors.initials: M
authors.lastname: Gharagozlou
authors.forename: Lida
authors.initials: L
authors.lastname: Atarod
authors.forename: Bahram Mirsaeid
authors.initials: BM
authors.lastname: Ghazi
authors.forename: Anna
authors.initials: A
authors.lastname: Isaeian
authors.forename: Maryam
authors.initials: M
authors.lastname: Mahmoudi
authors.forename: Kamran
authors.initials: K
authors.lastname: Abolmaali
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: Hedayat
authors.initials: H
authors.lastname: Akbari
authors.forename: Reza
authors.initials: R
authors.lastname: Amin
authors.forename: Abdolvahab
authors.initials: A
authors.lastname: Alborzi
authors.forename: Sara
authors.initials: S
authors.lastname: Kashef
authors.forename: Reza
authors.initials: R
authors.lastname: Farid
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: Abolhassan
authors.initials: A
authors.lastname: Farhoudi
mesh_terms: 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