title: Primary Immunodeficiency Diseases: An Opportunity in Pediatrics for Improving Patient Outcomes.
abstract: OBJECTIVE
  Primary immunodeficiency diseases (PIDDs) are caused by inherent deficits in immune defenses that result in abnormal susceptibility to infection. In most cases, early and appropriate diagnosis can improve patient outcomes. The objective of this study was to evaluate understanding, recognition, and diagnosis of PIDD among pediatricians.
  
  
  METHODS
  A mail survey sent to a sample of pediatricians obtained from the American Medical Association and American Osteopathic Association. Results were compared with a similar survey of specialists who are members of the American Academy of Asthma, Allergy and Immunology.
  
  
  RESULTS
  More than a third (35%) of pediatricians were uncomfortable with the recognition and diagnosis of PIDD despite 95% having ordered screening tests or referring patients to specialists to be evaluated for PIDD, and 77% having followed at leastone patient with PIDD. In all, 84% of pediatricians were unaware that professional guidelines for PIDD exist.
  
  
  CONCLUSIONS
  Patients with PIDD would benefit from improved recognition of the diseases by pediatricians in order to facilitate earlier diagnosis and optimize ongoing therapy.
authors.affiliation: Miami Children's Hospital, Miami, FL, USA vivian.hernandez-trujillo@mch.com.
authors.forename: Vivian P
authors.initials: VP
authors.lastname: Hernandez-Trujillo
authors.affiliation: Immune Deficiency Foundation, Towson, MD, USA.
authors.forename: Chris
authors.initials: C
authors.lastname: Scalchunes
authors.affiliation: Connecticut Asthma and Allergy Center, West Hartford, CT, USA University of Connecticut School of Medicine, Farmington, CT, USA Children's Hospital of Philadelphia, Philadelphia, PA, USA University of Pennsylvania School of Medicine, Philadelphia, PA, USA.
authors.forename: Hillary S
authors.initials: HS
authors.lastname: Hernandez-Trujillo
authors.affiliation: Immune Deficiency Foundation, Towson, MD, USA Abt SRBI Inc, Government Services Division, Silver Spring, MD, USA.
authors.forename: John
authors.initials: J
authors.lastname: Boyle
authors.affiliation: University of Washington School of Medicine, Seattle, WA, USA.
authors.forename: Paul
authors.initials: P
authors.lastname: Williams
authors.affiliation: Immune Deficiency Foundation, Towson, MD, USA.
authors.forename: Marcia
authors.initials: M
authors.lastname: Boyle
authors.affiliation: Texas Children's Hospital, Houston, TX, USA Baylor College of Medicine, Houston, TX, USA.
authors.forename: Jordan S
authors.initials: JS
authors.lastname: Orange
mesh_terms: D001291:Attitude of Health Personnel; D019538:Health Care Surveys / Q000706:statistics & numerical data*; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000628:therapy*; D010372:Pediatrics / Q000706:statistics & numerical data*; D010820:Physicians / Q000706:statistics & numerical data*; D010818:Practice Patterns, Physicians' / Q000706:statistics & numerical data*; D012017:Referral and Consultation; D014481:United States
keywords: diagnosis; pediatricians’ knowledge; primary immunodeficiency; treatment