[Waardenburg Syndrome: a review of literature and case reports]. / Revisione critica della letteratura sulla Sindrome di Waardenburg e presentazione di due casi clinici.

Waadernburg syndrome (WS) is an autosomal dominant disease clinically and genetically heterogeneous. It is characterized by non-progressive sensorineuronal hearing loss of variable degree and anomalous pigmentation (of the eyes, skin and stria vascularis of cochlear duct) caused by melanocytes absence. The syndrome is caused by mutations in genes that regulate the melanocytes differentiation from the neural crest during embriogenetic development. This study will review the literature on WS and explain more in detail the ocular findings in WS, while reporting new findings on two newly diagnosed cases.

Effectiveness of oral sodium cromoglycate (SCG) in preventing food allergy in children.

The efficacy of oral SCG in preventing food allergy symptoms in children was investigated. Ten children with cow's milk and/or egg IgE-mediated allergy were selected for this study. The subjects were challenged with the offending food before and after a seven-day pre-treatment period with oral SCG (30 mg/kg b.w. per day). Full protection was achieved in six out of eight children with cow's milk allergy and in four of the five children with egg allergy. The mode of action of SCG in the prevention of clinical manifestations of food allergy is discussed.

Results of a milk and/or egg free diet in children with atopic dermatitis.

Food allergy plays an important role in the pathogenesis of atopic dermatitis. Exclusion of eggs and milk from the diet of children during the first few months of life has been proven to significantly reduce the severity of eczema in children aged between 2 and 8 years. We studied the efficacy of exclusion of milk and/or egg from the diet of 59 children with severe atopic dermatitis. A detailed personal and family history was obtained. Total and specific IgE levels were determined on one hand, and intracutaneous tests were evaluated on the other. All allergens suspected of being implicated in the genesis of atopic dermatitis were eliminated from the diet. Clinical improvement was observed in 80% of cases after dietary exclusion. The younger children responded best, and the response to diet was not influenced by the severity of the eczema. No relation was observed between the family history of atopy and response to diet. Neither total nor specific IgE titers presented significant differences when the responder groups were compared.