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Oral Findings in Prader-Willi Syndrome – Case Report

Year: 2011
Type: article
Study design: Case Report
Abstract: The article describes the phenotypic features of Prader-Willi Syndrome (PWS) and their impact on the oral health status of a boy aged 11.7 years. Prader-Willi Syndrome is usually caused by a chromosomal aberration, i.e. a partial deletion of the long arm of the paternal chromosome 15q11-q13. The clinical picture of Prader-Willi Syndrome is complex and alters with age. It is mostly due to impaired hypothalamus function. The characteristic features include short stature; muscular hypotonia; hypopigmentation of the skin and hair; a triangular facies, frequently with a small mouth with a thin upper lip and down-turned corners; and diminished secretion of thick and sticky saliva. The case report focuses on the type of abnormal saliva secretion, injury lesions of the oral mucosa (caused by self-destructive behaviour), bacterial and fungal infection, bruxism and susceptibility to dental caries. The results of an orthodontic assessment are included, and the difficulties in preventative and therapeutic management caused by the behavioral and emotional problems typical of PWS are considered (Dent. Med. Probl. 2011, 48, 1, 103-107).
Language: English
Subfield: Genetics
Open Access status: diamond
OpenAlex ID: W2182952532
FWCI:
Cites: 18
Cited by: 3
Related to: 10