The oro-dental phenotype in Prader-Willi syndrome: a survey of 15 patients.
BACKGROUND
Prader-Willi syndrome (PWS) is a rare disorder caused by genetic defects in certain regions of chromosome 15q11-13. It is characterized by severe neonatal hypotonia and feeding problems, childhood-onset hyperphagia and obesity, short stature, facial dysmorphy, hypogonadism, learning and behavioural difficulties, and dental abnormalities.


OBJECTIVE
To describe the oro-dental phenotypic spectrum of patients with PWS.


METHODS
Fifteen PWS patients (3-35 years of age) being followed at the Centre for Human Genetics of the University Hospital of Leuven were examined at the dental clinic of the same institution. Medical information collected included age at diagnosis, body mass index (BMI) and level of cognitive functioning. Oral, clinical and radiological evaluations were performed. Caries experience (cavitation level), dental erosion and salivary flow rates were assessed.


RESULTS
The 15 patients had dmft/DMFT scores ranging from 0 to 28, while nine were cavity-free. Those with severe caries experience also presented advanced dental erosion. BMI ranged from 16 to 42.6. There was no association between BMI and caries experience or erosive tooth wear. The PWS patients in our survey presented with a more favourable oral health status than those in previous studies. This might be due to early diet management or better oral hygiene during childhood or both.
Bailleul-Forestier
Isabelle
I
Paediatric Dentistry, Garancière Hotel-Dieu Hospital, AP-HP France. ibailleul@noos.fr
Verhaeghe
Veroniek
V
Fryns
Jean-Pierre
JP
Vinckier
Frans
F
Declerck
Dominique
D
Vogels
Annick
A
D000293:Adolescent; D000328:Adult; D015992:Body Mass Index; D002648:Child; D002675:Child, Preschool; D002884:Chromosomes, Human, Pair 15; D004245:DMF Index; D017742:Dental Care for Chronically Ill / Q000379:methods*; D003731:Dental Caries / Q000453:epidemiology / Q000235:genetics; D019066:Facies; D005260:Female; D006801:Humans; D008297:Male; D010641:Phenotype; D011218:Prader-Willi Syndrome / Q000235:genetics*; D012472:Salivation / Q000502:physiology; D014077:Tooth Erosion / Q000453:epidemiology