title: Measurement of Health-Related Quality of Life in Primary Antibody-Deficient Patients.
abstract: BACKGROUND
  Primary immunodeficiency diseases are a group of disorders that result from a variety of defects of the immune system. Primary antibody deficiencies (PADs) are the most common forms of these disorders. Occurrence of recurrent infections, autoimmune diseases, cancers, and lymphoproliferative disorders is higher in PAD patients. Chronicity of these diseases, delayed diagnosis, inadequate treatment, and treatment side effects may affect the quality of life (QoL) of PAD patients. Evaluating QoL is important for patient care, understanding the burden of these diseases, and finding the patients' major health problems. We investigated the QoL in a group of PAD patients undergoing regular follow-up and treatment at the Children's Medical Center Hospital in Tehran, Iran.
  
  
  METHODS
  Seventy patients with a diagnosis of PAD in two age groups (younger and older than 18 years) were included. QoL was measured using PedsQL and SF-36 questionnaires. Correlation of demographic, clinical, and immunological parameters with QoL scores was assessed and patients' scores were compared with the normal population, using nonparametric tests of SPSS software.
  
  
  RESULTS
  Patients expressed significantly reduced scores in some mental and physical components. Patients with longer follow-up periods had higher scores in mental components but physical component scores were still low. There was no significant correlation between sex, age, and disease types with scores.
  
  
  CONCLUSIONS
  PAD patients had significantly lower scores in mental and physical components compared to normal population. By early diagnosis and long-term follow-up periods, we may be able to prevent complications and help patients to have a better QoL.
authors.affiliation: a Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center , Tehran University of Medical Sciences , Tehran , Iran.|b Students' Scientific Research Center (SSRC) , Tehran University of Medical Sciences , Tehran , Iran.
authors.forename: Bahar
authors.initials: B
authors.lastname: Ataeinia
authors.affiliation: c Iranian Institute for Health Sciences Research , Tehran , Iran.
authors.forename: Ali
authors.initials: A
authors.lastname: Montazeri
authors.affiliation: a Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center , Tehran University of Medical Sciences , Tehran , Iran.|d Department of Allergy and Clinical Immunology, Shahid Bahonar Hospital , Alborz University of Medical Sciences , Karaj , Iran.
authors.forename: Marzieh
authors.initials: M
authors.lastname: Tavakol
authors.affiliation: a Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center , Tehran University of Medical Sciences , Tehran , Iran.|e Department of Laboratory Medicine, Imam Hassan Mojtaba Hospital , Alborz University of Medical Sciences , Karaj , Iran.
authors.forename: Gholamreza
authors.initials: G
authors.lastname: Azizi
authors.affiliation: a Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center , Tehran University of Medical Sciences , Tehran , Iran.
authors.forename: Fatemeh
authors.initials: F
authors.lastname: Kiaee
authors.affiliation: a Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center , Tehran University of Medical Sciences , Tehran , Iran.
authors.forename: Naiimeh
authors.initials: N
authors.lastname: Tavakolinia
authors.affiliation: f School of Advanced Technologies in Medicine, Department of Medical Biotechnology , Tehran University of Medical Sciences , Tehran , Iran.
authors.forename: Babak
authors.initials: B
authors.lastname: Negahdari
authors.affiliation: g Department of Biomedical Engineering, Faculty of New Sciences and Technologies , University of Tehran , Tehran , Iran.
authors.forename: Javad
authors.initials: J
authors.lastname: Mohammadi
authors.affiliation: a Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center , Tehran University of Medical Sciences , Tehran , Iran.|h Division of Clinical Immunology, Department of Laboratory Medicine , Karolinska Institutet at the Karolinska University Hospital Huddinge , Stockholm , Sweden.
authors.forename: Hassan
authors.initials: H
authors.lastname: Abolhassani
authors.affiliation: a Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center , Tehran University of Medical Sciences , Tehran , Iran.|i Molecular Immunology Research Center, Pediatrics Center of Excellence, Children's Medical Center , Tehran University of Medical Sciences , Tehran , Iran.
authors.forename: Nima
authors.initials: N
authors.lastname: Rezaei
authors.affiliation: a Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center , Tehran University of Medical Sciences , Tehran , Iran.
authors.forename: Asghar
authors.initials: A
authors.lastname: Aghamohammadi
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000453:epidemiology* / Q000523:psychology; D007492:Iran / Q000453:epidemiology; D008297:Male; D008603:Mental Health; D005791:Patient Care; D011788:Quality of Life; D012959:Socioeconomic Factors; D011795:Surveys and Questionnaires; D055815:Young Adult
keywords: PedsQL; SF-36; primary antibody deficiency; quality of life