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Clinical Features That Identify Children With Primary Immunodeficiency Diseases

Lookup NU author(s): Professor Andrew GenneryORCiD, Dr Mary Slatter, Professor Andrew Cant

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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. Pediatrics 2011;127:810-816


Publication metadata

Author(s): Subbarayan A, Colarusso G, Hughes SM, Gennery AR, Slatter M, Cant AJ, Arkwright PD

Publication type: Article

Publication status: Published

Journal: Pediatrics

Year: 2011

Volume: 127

Issue: 5

Pages: 810-816

Print publication date: 01/05/2011

ISSN (print): 0031-4005

ISSN (electronic): 1098-4275

Publisher: American Academy of Pediatrics

URL: http://dx.doi.org/10.1542/peds.2010-3680

DOI: 10.1542/peds.2010-3680


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