Prevalence of atopic disorders and immunodeficiency in patients with ectodermal dysplasia syndromes.
Ann Allergy Asthma Immunol
; 108(6): 435-8, 2012 Jun.
Article
en En
| MEDLINE
| ID: mdl-22626597
ABSTRACT
BACKGROUND:
Ectodermal dysplasia (ED) syndromes are a diverse group of disorders that affect multiple ectodermally derived tissues. Small studies and case reports suggest an increase in atopy and primary immunodeficiencies (PIDs) among patients with ED syndromes.OBJECTIVE:
To determine the prevalence of clinical symptoms suggestive of atopy or immunodeficiency among a large cohort of children with ED syndromes.METHODS:
A 9-page questionnaire was mailed to families who were members of the National Foundation for Ectodermal Dysplasias. The surveys were completed by parents of children younger than 18 years with a diagnosis of an ED syndrome or carrier state. Portions of the questionnaire were adapted from previously validated questionnaires developed by the International Study of Asthma and Allergies in Childhood (ISAAC).RESULTS:
We received 347 completed questionnaires (41%). When compared with the 13- to 14-year-old children surveyed by ISAAC, we found both all-aged and age-matched children with ED syndromes, respectively, had significantly higher rates of asthma (32.2% and 37.2% vs 16.4%), rhinitis symptoms (76.1% and 78.3% vs 38.9%), and eczema (58.9% and 48.9% vs 8.2%). The prevalence of physician-diagnosed food allergies (20.7%) and PIDs (6.1%) in these ED patients also exceeded known rates in the general pediatric population.CONCLUSION:
This large-scale, retrospective study demonstrates a greater reported prevalence of symptoms suggestive of atopic disorders and PIDs among children with ED syndromes than the general pediatric population. A combination of genetic and environmental factors in ED syndromes may contribute to breaches of skin and mucosal barriers, permitting enhanced transmission and sensitization to irritants, allergens, and pathogens.
Texto completo:
1
Banco de datos:
MEDLINE
Asunto principal:
Displasia Ectodérmica
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Hipersensibilidad Inmediata
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Síndromes de Inmunodeficiencia
Tipo de estudio:
Etiology_studies
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Incidence_studies
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Observational_studies
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Prevalence_studies
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Risk_factors_studies
País/Región como asunto:
America do norte
Idioma:
En
Año:
2012
Tipo del documento:
Article