title: Long-term follow-up of ninety eight Iranian patients with primary immune deficiency in a single tertiary centre.
abstract: OBJECTIVE
  The aim was to describe the clinical manifestations, complications and long-term outcome of a cohort of Iranian patients with primary immune deficiency (PID).
  
  
  METHODS
  We retrospectively studied the demographic, clinical and immunological characteristics of the PID patients in a single tertiary centre, from January 1989 to July 2014. The patients were classified according to the International Union of Immunological Societies Expert Committee on PID.
  
  
  RESULTS
  98 patients were diagnosed with and followed-up for 15 disorders. The mean age at onset and diagnosis and the diagnostic delay were 8±10, 14.2±13.1 and 6.1±7 years, respectively. Parental consanguinity rate was 57%. Predominantly Antibody Deficiency was the most common diagnosis (n=63), followed by congenital defects of phagocytes (n=16), combined immunodeficiencies (n=12), well defined syndromes (n=4) and defects in innate immunity (n=3). Recurrent sinopulmonary infection was the most common presentation. Active infections were treated appropriately, in addition to prophylactic therapy with IVIG and antimicrobials. Not all the patients were compliant with prophylactic regimens due to cost and unavailability. One SCID patient underwent successful bone marrow transplantation. The total mortality rate was 19% during the follow-up period (7.8±7.6 years). The mean age of living patients at the time of study was 23±11.7 years.
  
  
  CONCLUSIONS
  Physicians awareness of PID has been rising dramatically in Iran, ensuring an increasing number of patients being diagnosed and treated. More effective treatment services, including health insurance coverage and drug availability are needed to improve the outcome of PID patients.
authors.affiliation: Department of Allergy and Clinical Immunology, Rasool-e-Akram Hospital, Iran University of Medical Sciences (IUMS), Tehran, Iran.
authors.forename: M
authors.initials: M
authors.lastname: Nabavi
authors.affiliation: Department of Allergy and Clinical Immunology, Rasool-e-Akram Hospital, Iran University of Medical Sciences (IUMS), Tehran, Iran.
authors.forename: S
authors.initials: S
authors.lastname: Arshi
authors.affiliation: Department of Allergy and Clinical Immunology, Rasool-e-Akram Hospital, Iran University of Medical Sciences (IUMS), Tehran, Iran.
authors.forename: M H
authors.initials: MH
authors.lastname: Bemanian
authors.affiliation: Research Center for Immunodeficiency, Children's Medical Center, Tehran University of Medical Sciences (TUMS), Tehran, Iran.
authors.forename: A
authors.initials: A
authors.lastname: Aghamohammadi
authors.affiliation: Department of Internal Medicine, Division of Infectious Diseases and Clinical Immunology, National Research Institute of Tuberculosis and Lung Diseases, Masih-e-Daneshvari Hospital, Shaheed Beheshti University of Medical Sciences (SBUMS), Tehran, Iran.
authors.forename: D
authors.initials: D
authors.lastname: Mansouri
authors.affiliation: Division of Immunology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
authors.forename: M
authors.initials: M
authors.lastname: Hedayat
authors.affiliation: Department of Infectious Diseases, Ali-Asghar Hospital, IUMS, Tehran, Iran.
authors.forename: A
authors.initials: A
authors.lastname: Nateghian
authors.affiliation: Research Center of Pediatric Infectious Diseases, Rasool-e-Akram Hospital, IUMS, Tehran, Iran.
authors.forename: S
authors.initials: S
authors.lastname: Noorbakhsh
authors.affiliation: Research Center of Pediatric Infectious Diseases, Rasool-e-Akram Hospital, IUMS, Tehran, Iran.
authors.forename: F
authors.initials: F
authors.lastname: Ehsanipour
authors.affiliation: Department of Pediatric Oncology, Rasool-e-Akram Hospital, IUMS, Tehran, Iran.
authors.forename: M
authors.initials: M
authors.lastname: Faranoush
authors.affiliation: Digestive Oncology Research Center, Digestive Disease Research Institute, TUMS, Tehran, Iran.
authors.forename: R
authors.initials: R
authors.lastname: Shakeri
authors.affiliation: Department of Immunology, Mofid Children's Hospital, SBUMS, Tehran, Iran.
authors.forename: M
authors.initials: M
authors.lastname: Mesdaghi
authors.affiliation: Department of Allergy and Clinical Immunology, Rasool-e-Akram Hospital, Iran University of Medical Sciences (IUMS), Tehran, Iran.
authors.forename: B
authors.initials: B
authors.lastname: Taghvaei
authors.affiliation: Department of Allergy and Clinical Immunology, Rasool-e-Akram Hospital, Iran University of Medical Sciences (IUMS), Tehran, Iran; Research Center of Otolaryngology and Head & Neck Surgery, IUMS, Tehran, Iran.
authors.forename: B
authors.initials: B
authors.lastname: Ghalebaghi
authors.affiliation: Department of Allergy and Clinical Immunology, Mofid Hospital, SBUMS, Tehran, Iran.
authors.forename: D
authors.initials: D
authors.lastname: Babaie
authors.affiliation: Department of Allergy and Clinical Immunology, Rasool-e-Akram Hospital, Iran University of Medical Sciences (IUMS), Tehran, Iran.
authors.forename: A
authors.initials: A
authors.lastname: Bahrami
authors.affiliation: Department of Allergy and Clinical Immunology, Rasool-e-Akram Hospital, Iran University of Medical Sciences (IUMS), Tehran, Iran.
authors.forename: M
authors.initials: M
authors.lastname: Fallahpour
authors.affiliation: Allergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
authors.forename: H
authors.initials: H
authors.lastname: Esmaeilzadeh
authors.affiliation: Hematology Oncology and Stem Cell Transplantation Research Center, TUMS, Iran.
authors.forename: A
authors.initials: A
authors.lastname: Ali Hamidieh
authors.affiliation: Department of Allergy and Clinical Immunology, Rasool-e-Akram Hospital, Iran University of Medical Sciences (IUMS), Tehran, Iran.
authors.forename: M
authors.initials: M
authors.lastname: Rekabi
authors.affiliation: Department of Allergy and Clinical Immunology, Rasool-e-Akram Hospital, Iran University of Medical Sciences (IUMS), Tehran, Iran.
authors.forename: J
authors.initials: J
authors.lastname: Ahmadian
authors.affiliation: Department of Allergy and Clinical Immunology, Rasool-e-Akram Hospital, Iran University of Medical Sciences (IUMS), Tehran, Iran.
authors.forename: N
authors.initials: N
authors.lastname: Eslami
authors.affiliation: Department of Allergy and Clinical Immunology, Rasool-e-Akram Hospital, Iran University of Medical Sciences (IUMS), Tehran, Iran.
authors.forename: S
authors.initials: S
authors.lastname: Shokri
authors.affiliation: Antimicrobial Resistance Research Center, Rasool-e-Akram Hospital, IUMS, Tehran, Iran.
authors.forename: M
authors.initials: M
authors.lastname: Afshar
authors.affiliation: Department of Allergy and Clinical Immunology, Rasool-e-Akram Hospital, Iran University of Medical Sciences (IUMS), Tehran, Iran.
authors.forename: F
authors.initials: F
authors.lastname: Jalali
authors.affiliation: Department of Allergy and Clinical Immunology, Rasool-e-Akram Hospital, Iran University of Medical Sciences (IUMS), Tehran, Iran.
authors.forename: N
authors.initials: N
authors.lastname: Akbarpour
authors.affiliation: Department of Allergy and Clinical Immunology, Rasool-e-Akram Hospital, Iran University of Medical Sciences (IUMS), Tehran, Iran. Electronic address: molatefi.r@tak.iums.ac.ir.
authors.forename: R
authors.initials: R
authors.lastname: Molatefi
authors.affiliation: Research Center for Immunodeficiency, Children's Medical Center, Tehran University of Medical Sciences (TUMS), Tehran, Iran; Molecular Immunology Research Center, and Department of Immunology, School of Medicine, TUMS, Tehran, Iran; Universal Scientific Education and Research Network (USERN), Tehran, Iran.
authors.forename: N
authors.initials: N
authors.lastname: Rezaei
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D003241:Consanguinity; D057210:Delayed Diagnosis; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage / Q000627:therapeutic use*; D007153:Immunologic Deficiency Syndromes / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology; D007155:Immunologic Factors / Q000008:administration & dosage / Q000627:therapeutic use*; D007492:Iran / Q000453:epidemiology; D008297:Male; D012141:Respiratory Tract Infections / Q000188:drug therapy / Q000453:epidemiology* / Q000209:etiology / Q000517:prevention & control; D012189:Retrospective Studies; D062606:Tertiary Care Centers / Q000706:statistics & numerical data; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors
keywords: Complications; Consanguinity; Follow-up; Iran; Manifestations; Mortality; Primary immune deficiency