ProblemPrimary 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.MethodIn this study, the records 515 Iranian PiD patients were reviewed during a 25‐year period.ResultsThe 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, seletive IgG class deficiencies, chronic granulomatous disease, and Schwachman syndrome.ConclusionsIt 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.

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.

N Rezaei, A Aghamohammadi, A Farhoudi, M Moein, Z Pourpak, K Abolmaali, M Movahedi, M Gharagozlou, B MirSaeid Ghazi, M Mahmoudi, D Mansouri, S Arshi, N Javaher Tarash, H Akbari, R Sherkat, M R Farid, A Hashemzadeh, I Mohammadzadeh, R Amin, S Kashef, A Alborzi, A Karimi Tehran University of Medical Sciences, Children’s Medical Center, Department of Immunology, Allergy and Asthma, Tehran, Islamic Republic of Iran, Beheshti University of Medical Sciences, Daneshvari Hospital, Department of Infectious Disease, Tehran, Islamic Republic of Iran, Iran University of Medical Sciences, Alrasoul Hospital, Department of Immunology and Allergy, Tehran, Islamic Republic of Iran, Esfehan University of Medical Sciences, Alzahara Hospital, Department of Clinical Paediatric Immunology, Esfehan, Islamic Republic of Iran, Mashhad University of Medical Sciences, Department of Immunology and Allergy, Mashhad, Islamic Republic of Iran, Babol University of Medical Sciences, Department of Clinical Pediatric Immunology, Babol, Islamic Republic of Iran, Shiraz University of Medical Sciences, Namazi Hospital, Department of Immunology and Allergy, Shiraz, Islamic Republic of Iran