title: Clinical features that identify children with primary immunodeficiency diseases.
abstract: BACKGROUND
  The 10 warning signs of primary immunodeficiency diseases (PID) have been promoted by various organizations in Europe and the United States to predict PID. However, the ability of these warning signs to identify children with PID has not been rigorously tested.
  
  
  OBJECTIVE
  The main goal of this study was to determine the effectiveness of these 10 warning signs in predicting defined PID among children who presented to 2 tertiary pediatric immunodeficiency centers in the north of England.
  
  
  METHODS
  A retrospective survey of 563 children who presented to 2 pediatric immunodeficiency centers was undertaken. The clinical records of 430 patients with a defined PID and 133 patients for whom detailed investigations failed to establish a specific PID were reviewed.
  
  
  RESULTS
  Overall, 96% of the children with PID were referred by hospital clinicians. The strongest identifiers of PID were a family history of immunodeficiency disease in addition to use of intravenous antibiotics for sepsis in children with neutrophil PID and failure to thrive in children with T-lymphocyte PID. With these 3 signs, 96% of patients with neutrophil and complement deficiencies and 89% of children with T-lymphocyte immunodeficiencies could be identified correctly. Family history was the only warning sign that identified children with B-lymphocyte PID.
  
  
  CONCLUSIONS
  PID awareness initiatives should be targeted at hospital pediatricians and families with a history of PID rather than the general public. Our results provide the general pediatrician with a simple refinement of 10 warning signs for identifying children with underlying immunodeficiency diseases.
authors.affiliation: Department of Paediatric Allergy and Immunology, Royal Manchester Children's Hospital, University of Manchester, Oxford Road, Manchester M13 9WL, United Kingdom.
authors.forename: Anbezhil
authors.initials: A
authors.lastname: Subbarayan
authors.forename: Gloria
authors.initials: G
authors.lastname: Colarusso
authors.forename: Stephen M
authors.initials: SM
authors.lastname: Hughes
authors.forename: Andrew R
authors.initials: AR
authors.lastname: Gennery
authors.forename: Mary
authors.initials: M
authors.lastname: Slatter
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Cant
authors.forename: Peter D
authors.initials: PD
authors.lastname: Arkwright
mesh_terms: D000900:Anti-Bacterial Agents / Q000627:therapeutic use*; D001424:Bacterial Infections / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology; D001774:Blood Chemical Analysis; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006403:Hematologic Tests; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000175:diagnosis*; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008487:Medical History Taking; D008499:Medical Records; D011237:Predictive Value of Tests; D012008:Recurrence; D012017:Referral and Consultation; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D017211:Treatment Failure; D006113:United Kingdom
chemical_list: D000900:Anti-Bacterial Agents