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Keywords

Rett syndrome, Eating/swallowing functions, Aging, Nutritional status, Respiratory status

Abstract

This study was conducted to clarify the actual status of oral symptoms related to the systemic and eating/swallowing functions, and the effect of ageing for those conditions in patients with Rett syndrome. The subjects were 38 females aged from 4 to 63 years diagnosed with Rett syndrome. The examination items were age, nutritional status, gross motor functions, history of epileptic seizures, antiepileptic drugs use, history of pneumonia, respiratory status (apnea, hyperventilation, and aerophagia), ingested food type, eating/swallowing functions, dysphagia symptom, occlusal condition, eating independence level, palate shape, and bruxism. Using Pearson's correlation coefficient, no significant correlation between age and nutritional status was observed (Rohrer index: p=0.12, BMI: p=0.22). When the other inter-item relationships were evaluated by the x square test and Fisher's exact test, a significant correlation was recognized between gross motor functions and oral functions (p < 0.01 ), between gross motor functions and swallowing function (p < 0.05), and between respiratory status and oral functions (p < 0.05). The investigation of the characteristics of patients with Rett syndrome suggested that decreased gross motor functions, such as gait function, and the appearance of respiratory status might herald the development of eating/swallowing dysfunction.

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