title: Primary immunodeficiency in Iran: first report of the National Registry of PID in Children and Adults.
abstract: Epidemiological studies have shown wide geographical and racial variation in the prevalence and patterns of immunodeficiency disorders. To determine the frequency of primary immunodeficiencies (PID) in Iran, the Iranian Primary Immunodeficiency Registry (IPIDR) was organized in 1999. We extracted the patient's data, by using a uniform questionnaire from their hospital records. The diagnosis of patients was based on WHO criteria. By now, 440 patients with PID, who were observed during a period of 20 years, have been registered in our registry. Among these patients, the following frequencies were found: predominantly antibody deficiency in 45.9% of patients (n = 202), phagocytic disorders in 29.09% (n = 128), T-cell disorders in 24.31% (n = 107), and complement deficiencies in 0.68% (n = 3). Common variable immunodeficiency was the most frequent disorder (n = 98), followed by chronic granulomatous disease (n = 86), ataxia telangiectasia (n = 48), x-linked agammaglobulinemia (n = 45), selective IgA deficiency (n = 42), combined immunodeficiency (n = 15), and severe combined immunodeficiency (n = 14). This study revealed that antibody deficiencies is the most frequently diagnosed primary immunodeficiency disorder in our patients, which is similar to that observed in other registries. A comparative study shows some differences between our results and other registries.
authors.affiliation: Department of Clinical Pediatric Immunology, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Asghar
authors.initials: A
authors.lastname: Aghamohammadi
authors.forename: Mosafa
authors.initials: M
authors.lastname: Moein
authors.forename: Abolhasan
authors.initials: A
authors.lastname: Farhoudi
authors.forename: Zahra
authors.initials: Z
authors.lastname: Pourpak
authors.forename: Nima
authors.initials: N
authors.lastname: Rezaei
authors.forename: Kamran
authors.initials: K
authors.lastname: Abolmaali
authors.forename: Masoud
authors.initials: M
authors.lastname: Movahedi
authors.forename: Mohammad
authors.initials: M
authors.lastname: Gharagozlou
authors.forename: Bahram Mir Saeid
authors.initials: BM
authors.lastname: Ghazi
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: Trash
authors.forename: Hedayatallah
authors.initials: H
authors.lastname: Akbari
authors.forename: Roya
authors.initials: R
authors.lastname: Sherkat
authors.forename: Reza Farid
authors.initials: RF
authors.lastname: Hosayni
authors.forename: Ahmad
authors.initials: A
authors.lastname: Hashemzadeh
authors.forename: Iraj
authors.initials: I
authors.lastname: Mohammadzadeh
authors.forename: Reza
authors.initials: R
authors.lastname: Amin
authors.forename: Sara
authors.initials: S
authors.lastname: Kashef
authors.forename: Abdalvahab
authors.initials: A
authors.lastname: Alborzi
authors.forename: Abdallah
authors.initials: A
authors.lastname: Karimi
authors.forename: Hosaynali
authors.initials: H
authors.lastname: Khazaei
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications / Q000453:epidemiology*; D007223:Infant; D007231:Infant, Newborn; D007239:Infections / Q000209:etiology; D007492:Iran / Q000453:epidemiology; D008297:Male; D012042:Registries; D011795:Surveys and Questionnaires