Cargando…
Clinical Predictors of Primary Immunodeficiency Diseases in Children
PURPOSE: To promote awareness of primary immunodeficiency (PID), the "10 warning signs" of PID and an immunodeficiency-related (IDR) score were developed. However, their efficiency in identifying PID cases was not sufficiently evaluated in clinical practice. The objective of this study was...
Autores principales: | , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The Korean Academy of Asthma, Allergy and Clinical Immunology; The Korean Academy of Pediatric Allergy and Respiratory Disease
2013
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3579097/ https://www.ncbi.nlm.nih.gov/pubmed/23450209 http://dx.doi.org/10.4168/aair.2013.5.2.88 |
_version_ | 1782260089164398592 |
---|---|
author | Reda, Shereen M. El-Ghoneimy, Dalia H. Afifi, Hanaa M. |
author_facet | Reda, Shereen M. El-Ghoneimy, Dalia H. Afifi, Hanaa M. |
author_sort | Reda, Shereen M. |
collection | PubMed |
description | PURPOSE: To promote awareness of primary immunodeficiency (PID), the "10 warning signs" of PID and an immunodeficiency-related (IDR) score were developed. However, their efficiency in identifying PID cases was not sufficiently evaluated in clinical practice. The objective of this study was to test the validity of the 10 warning signs and IDR score in identifying PID among children with recurrent infections at a tertiary pediatric hospital in Egypt. METHODS: A retrospective analysis of the medical records of 204 patients was performed. Of these patients, 92 had defined PID diseases and 112 were considered non-PID cases because investigations were inconclusive. RESULTS: Demonstrating two warning signs and an IDR score of 6 led to sensitivities of 94 and 66%, respectively, and specificities of 64 and 75%, respectively, in identifying PID cases. The strongest predictor of PID was family history that, if combined with the need for intravenous antibiotics, recurrent deep-seated infections, and failure to thrive, could identify 81% of PID patients. A family history of PID, sibling death, and/or parental consanguinity would predict 92% of combined immunodeficiencies, 92% of phagocyte defects, 87% of well-identified immunodeficiency syndromes, and 84% of antibody deficiency if the need for intravenous antibiotics is considered in the latter. CONCLUSIONS: The 10 warning signs and IDR score do not aid in an early diagnosis of severe PID. Educational campaigns should target pediatricians aiming to increase PID awareness and to address family history of PID, parental consanguinity, and previous sibling death as key predictors of PID in communities with a high prevalence of consanguineous marriages. |
format | Online Article Text |
id | pubmed-3579097 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2013 |
publisher | The Korean Academy of Asthma, Allergy and Clinical Immunology; The Korean Academy of Pediatric Allergy and Respiratory Disease |
record_format | MEDLINE/PubMed |
spelling | pubmed-35790972013-03-01 Clinical Predictors of Primary Immunodeficiency Diseases in Children Reda, Shereen M. El-Ghoneimy, Dalia H. Afifi, Hanaa M. Allergy Asthma Immunol Res Original Article PURPOSE: To promote awareness of primary immunodeficiency (PID), the "10 warning signs" of PID and an immunodeficiency-related (IDR) score were developed. However, their efficiency in identifying PID cases was not sufficiently evaluated in clinical practice. The objective of this study was to test the validity of the 10 warning signs and IDR score in identifying PID among children with recurrent infections at a tertiary pediatric hospital in Egypt. METHODS: A retrospective analysis of the medical records of 204 patients was performed. Of these patients, 92 had defined PID diseases and 112 were considered non-PID cases because investigations were inconclusive. RESULTS: Demonstrating two warning signs and an IDR score of 6 led to sensitivities of 94 and 66%, respectively, and specificities of 64 and 75%, respectively, in identifying PID cases. The strongest predictor of PID was family history that, if combined with the need for intravenous antibiotics, recurrent deep-seated infections, and failure to thrive, could identify 81% of PID patients. A family history of PID, sibling death, and/or parental consanguinity would predict 92% of combined immunodeficiencies, 92% of phagocyte defects, 87% of well-identified immunodeficiency syndromes, and 84% of antibody deficiency if the need for intravenous antibiotics is considered in the latter. CONCLUSIONS: The 10 warning signs and IDR score do not aid in an early diagnosis of severe PID. Educational campaigns should target pediatricians aiming to increase PID awareness and to address family history of PID, parental consanguinity, and previous sibling death as key predictors of PID in communities with a high prevalence of consanguineous marriages. The Korean Academy of Asthma, Allergy and Clinical Immunology; The Korean Academy of Pediatric Allergy and Respiratory Disease 2013-03 2012-11-02 /pmc/articles/PMC3579097/ /pubmed/23450209 http://dx.doi.org/10.4168/aair.2013.5.2.88 Text en Copyright © 2013 The Korean Academy of Asthma, Allergy and Clinical Immunology • The Korean Academy of Pediatric Allergy and Respiratory Disease http://creativecommons.org/licenses/by-nc/3.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Original Article Reda, Shereen M. El-Ghoneimy, Dalia H. Afifi, Hanaa M. Clinical Predictors of Primary Immunodeficiency Diseases in Children |
title | Clinical Predictors of Primary Immunodeficiency Diseases in Children |
title_full | Clinical Predictors of Primary Immunodeficiency Diseases in Children |
title_fullStr | Clinical Predictors of Primary Immunodeficiency Diseases in Children |
title_full_unstemmed | Clinical Predictors of Primary Immunodeficiency Diseases in Children |
title_short | Clinical Predictors of Primary Immunodeficiency Diseases in Children |
title_sort | clinical predictors of primary immunodeficiency diseases in children |
topic | Original Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3579097/ https://www.ncbi.nlm.nih.gov/pubmed/23450209 http://dx.doi.org/10.4168/aair.2013.5.2.88 |
work_keys_str_mv | AT redashereenm clinicalpredictorsofprimaryimmunodeficiencydiseasesinchildren AT elghoneimydaliah clinicalpredictorsofprimaryimmunodeficiencydiseasesinchildren AT afifihanaam clinicalpredictorsofprimaryimmunodeficiencydiseasesinchildren |