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Title: A survey of children affected by ectomermal dysplasia syndromes shows an increased prevalence of atopic disorders and immune deficiency

Author
item MOTIL, K - Children'S Nutrition Research Center (CNRC)

Submitted to: Annals of Allergy, Asthma and Immunology
Publication Type: Abstract Only
Publication Acceptance Date: 12/1/2008
Publication Date: 1/1/2009
Citation: Motil, K.J. 2009. A survey of children affected by ectomermal dysplasia syndromes shows an increased prevalence of atopic disorders and immune deficiency [abstract]. Annals of Allergy, Asthma and Immunology. 102(1):A104-105.

Interpretive Summary:

Technical Abstract: Ectodermal dysplasia (ED) syndromes are rare genetic disorders that affect the development of tissues derived from the embryonic ectoderm. Studies and anecdotal experience have indicated that atopic disorders (AD) and immune deficiencies (ID) may be associated with ED in children. Some ED genotypes have been associated with ID. The purpose of this study is to assess the prevalence of AD and ID in a large population of children with ED. Parents/guardians of children affected by ED syndromes completed a questionnaire about their children's personal and family history of ED, AD, and ID. The investigators developed the questionnaire, with portions adopted from the validated International Study of Asthma and Allergies in Childhood (ISAAC) questionnaire. Surveys were mailed to parents/guardians of 838 children in the membership directory of the National Foundation for Ectodermal Dysplasias. Comparison of prevalence rates for allergic rhinoconjunctivitis, asthma, and eczema were compared to published rates for ISAAC participants from North America using Chi-square tests. Results were compared for all respondents, and specifically for 13- to 14-year olds for which there is ISAAC data. Rates for food allergy and ID were compared to published reports. Responses were received from 347 of 838 mailed surveys (41%). Prevalence rates for AD were significantly higher for all ED respondents vs. 13-14 year old ED respondents vs. the general population (ever wheeze 50% vs. 58% vs. 33%; asthma diagnosis 31% vs. 37% vs. 16%; intermittent pruritic rash 57% vs. 54% vs. 17%; eczema diagnosis 57% vs. 49% vs. 8%; all p< 0.0001). Food allergy was reported as 34% vs. 4-8% in the general population. ID were reported by 4.9% vs. 0.05% in the general population. In addition, 1.4% of ED subjects reported having received IVIG. The rate of AD and ID is higher in this population of children with ED than the general population. Possible mechanisms include increased antigen absorption and presentation through the affected tissue or alterations of inflammatory responses in ED patients. Further evaluation of AD and ID in children with ED is warranted.