OBJECTIVESThe present study aimed to define the framework of an environment conducive to the well-being of children with intellectually disability (CID).

METHODSA questionnaire composed of 31 items was developed through literature review. Then a 2-round Delphi survey was conducted with 3 different panels: health professionals (HPs), parents of CID, and teachers. The participants were asked to rate each item, select and rank the 10 most important items, and suggest additional ones.

RESULTSA total of 71 participants responded to the first round: 24 HPs, 22 parents, and 25 teachers. In the second round the overall response rate was 83%. At the end of the exercise, 12 items reached global consensus, i.e., in all groups. Only 5 items were ranked as most important by all groups: attitudes of family members at home; attitudes of HPs and teachers; support from family members at home; support at school (classmates and teachers); and government policies. Nevertheless, the panelists' views diverged on the remaining items. Several additional elements were suggested.

CONCLUSIONSThe views of HPs, teachers, and parents are complementary for the improvement of quality of life (QOL) of CID. The present findings will be used as a basis for the development of an instrument to assess the living environment of CID.

Anhidrotic ectodermal dysplasia (AED) is a rare hereditary disorder with a triad of sparse hair, dental hypoplasia, and anhidrosis. Here we report a case of AED with food allergy and atopic eczema. The patient was a 11-month-old boy admitted to our hospital with pyrexia for 2 weeks. He presented with a history of dry skin, eczema, and food allergy to egg. On clinical examination, his body temperature was 38.8°C, with dry skin and eczema almost all over the body, sparse eyebrows, and scalp hair. Laboratory investigations and physical examination did not show any evidence of infection. Radioallergosorbent test was positive to egg yolk, egg white, ovomucoid, milk, house dust, and house dust mite. As the child did not sweat despite the high fever, we performed the sweat test which revealed a total lack of sweat glands. Genetic examination revealed a mutation of the EDA gene and he was diagnosed as AED. His pyrexia improved upon cooling with ice and fan. His mother had lost 8 teeth and her sweat test demonstrated low sweating, suggestive of her being a carrier of AED. Atopy and immune deficiencies have been shown to have a higher prevalence in patients with AED. Disruption of the skin barrier in patients with AED make them more prone to allergic diseases such as atopic eczema, bronchial asthma, allergic rhinitis and food allergy. Careful assessment of the familial history is essential to differentiate AED when examining patients with pyrexia of unknown origin and comorbid allergic diseases.
Asthma ; Body Temperature ; Child ; Dermatitis, Atopic ; Dust ; Ectodermal Dysplasia ; Eczema ; Egg White ; Egg Yolk ; Eyebrows ; Fever ; Food Hypersensitivity ; Hair ; Humans ; Hypohidrosis ; Ice ; Infant ; Male ; Milk ; Mothers ; Ovomucin ; Ovum ; Physical Examination ; Prevalence ; Pyroglyphidae ; Radioallergosorbent Test ; Rhinitis, Allergic ; Scalp ; Skin ; Sweat ; Sweat Glands ; Sweating ; Tooth