title: Impact of delayed diagnosis in children with primary antibody deficiencies.
abstract: BACKGROUND
  Primary antibody deficiencies (PADs) are heterogeneous group of disorders, characterized by hypogammaglobulinemia and increased susceptibility to recurrent infections. To evaluate the diagnostic delay in Iranian PADs in association with their infections, we scored such manifestations to find an association between such delay and the scoring system.
  
  
  METHODS
  Forty-eight patients with PADs, who were referred to our center during a 25-year period, were enrolled in this study. Each episode of infection, before making the PADs diagnosis, got a score of 5 or 10 based on the severity of infections.
  
  
  RESULTS
  The diagnosis was made with median delay of 34.5 months, when the patients had mean score of 94.48 ± 52.89. There was a significant direct association between this scoring system and delay diagnosis. The score of 50 was considered as the cutoff point in our patient group. In this score, the suspicions to PADs in more than 90% of patients true positively lead to diagnosis of PADs.
  
  
  CONCLUSIONS
  Although diagnosis delay significantly decreased over time, PADs still continue to be diagnosed late. Based on the results of this study, the assessment of immune system should be performed in the patients with 50 total score or about 25 score per year.
authors.affiliation: 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
authors.forename: Ahmad
authors.initials: A
authors.lastname: Bahrami
authors.forename: Setareh
authors.initials: S
authors.lastname: Mamishi
authors.forename: Babak
authors.initials: B
authors.lastname: Mohammadi
authors.forename: Hassan
authors.initials: H
authors.lastname: Abolhassani
authors.forename: Nima
authors.initials: N
authors.lastname: Parvaneh
authors.forename: Nima
authors.initials: N
authors.lastname: Rezaei
mesh_terms: D000293:Adolescent; D000361:Agammaglobulinemia / Q000175:diagnosis* / Q000276:immunology; D000906:Antibodies / Q000097:blood*; D002648:Child; D002675:Child, Preschool; D057210:Delayed Diagnosis / Q000009:adverse effects*; D004198:Disease Susceptibility; D005190:Family; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000175:diagnosis* / Q000235:genetics / Q000276:immunology; D007223:Infant; D007249:Inflammation / Q000276:immunology; D008297:Male
chemical_list: D000906:Antibodies