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Oral disorders in children with Prader-Willi syndrome: a case control study

Oral disorders in children with Prader-Willi syndrome: a case control study

Feb 2020, Munne-Miralves, Brunet-Llobet et. Al, Orphanet J Rare Dis

Summarized by: Sara Supriyatno

Prader-Willi Syndrome (PWS) is a complex genetic disorder, caused by deletion of paternal genes located in the region 15q11-13, has a prevalence of 3.1:100,000 newborns and affects sex and races equally. The condition affects individuals in a multisystemic way, with manifestations including hypotonia, developmental impairment, endocrine abnormalities, and hyperphagia. Dietary issues associated with this condition result in increased risk in dental and oral pathologies. A major oral manifestation of PWS is low and thickened salivary flow, due to the atrophy of salivary gland. A recent study by Munnè-Miralvès, et al. looked at the quantity and quality of stimulated salivary secretion of the affected individuals. Patients with PWS presented a mean of 0.474ml/min of salivary flow with a pH of 6.15, compared to the controls who presented with a mean of 0.848ml/min flow with a pH of 7.53. The decrease in salivary flow with a more acidic pH, in combination with poor oral hygiene and high carbohydrate food intakes, results in increased risk in caries and periodontal diseases in these pediatric patients. Therefore, early preventative dental care as a part of a multidisciplinary therapeutic approach is essential to improve the patient’s quality of life.

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