Oral mucosa in children with Prader-Willi syndrome

Abstract

Prader-Willi syndrome is a genetic disorder. Abnormal saliva secretion, emotional and behaviour problems, may affect the health status of the oral mucousa.

Objectives: To assess the impact of self-destructive behaviour and abnormal saliva secretion on the oral mucosa in children with Prader-Willi syndrome (PWS).

Materials and methods: Fifteen PWS's children (mean age 9.8 ± 4.4 years) and 15 healthy children (mean age 11.5 ± 3.5 years) were assessed for self-destructive behaviours, such as picking at the skin, physical and chemical saliva characteristics, mycology, and the clinical status of the oral mucosa.

Results: Picking at the skin was only in children with PWS (n = 12). In contrast to the control group, the moistening rate of the lower lip mucosa was slower, and the mean pH of the resting saliva was reduced in the affected subjects. Sticky frothy or frothy saliva, decreased secretion rate of the stimulated saliva, and a reduced buffer capacity were more frequently in PWS's children; Candida spp. and oral candidiasis were also more common. Injurious lesions in the oral mucosa were found in one control child, and in eight PWS's subjects. In affected children, the lesions were concurrent with picking at the skin. A statistical correlation was noted between the presence of Candida spp. and oral candidiasis, and unfavourable saliva properties, and between injurious lesions and a slow moistening rate of the lower lip mucosa, and oral candidiasis.

Conclusions: Abnormal saliva secretion and self-destructive behaviours in children with Prader-Willi syndrome predispose them to injurious lesions in the oral mucosa, and possibly, to oral candidiosis.