Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2020 Feb 10;15(1):43.
doi: 10.1186/s13023-020-1326-8.

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

Carla Munné-Miralvés  1 , Lluís Brunet-Llobet  2   3 , Abel Cahuana-Cárdenas  1 , Sergi Torné-Durán  4 , Jaume Miranda-Rius  1   5   4 , Alejandro Rivera-Baró  1   5
Affiliations

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

Carla Munné-Miralvés et al. Orphanet J Rare Dis. .

Abstract

Introduction: Prader-Willi Syndrome (PWS) is a genetic disorder caused by the lack of expression of certain paternal genes located on chromosome 15q11-q13. This anomaly causes cognitive, neurological and endocrine abnormalities, among which one of the most important is hyperphagia. The aim of this study was to assess the oral health of children with PWA and to establish preventive criteria.

Results: Thirty patients with PWS (mean age 10.2 years) and 30 age- and gender-matched controls were included in the study. Twenty-six patients with PWS(86.6%) followed dietary treatment prescribed by their endocrinologist. Individuals with PWS had a mean caries index of 53.3% and Decayed Missing Filled teeth (DMFT) index 2.5, and 53.3% had gingivitis, in the control group the respective figures were 43.3%, 0.93, and 60%. Only the DMFT index (p 0.017) presented significant differences. Regarding stimulated salivary secretion, patients with PWS presented a mean of 0.475 ml/min with a pH of 6.15, while controls presented a mean of 0.848 ml/min with a pH of 7.53; the differences between the groups were statistically significant in both cases (p 0.032 and p 0.0001 respectively). The population with PWS presented a higher plaque index (> 2) than their healthy peers, but the differences were not significant.

Conclusion: Pediatric patients with Prader-Willi syndrome have an increased risk of caries and gingivitis. The children with this syndrome have a decreased salivary flow and a more acidic salivary pH. In these patients, dental care is an essential part of their multidisciplinary medical treatment.

Keywords: Caries index (CI); Hyperphagia; Plaque index (PI); Prader-Willi syndrome (PWS); Salivary alteration.

PubMed Disclaimer

Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Hypotonia in a newborn with PWS
Fig. 2
Fig. 2
Physical characteristics of PWS
Fig. 3
Fig. 3
a.b/ Deciduous dentition. Observe the areas of attrition with severe incisal wear, and erosions present in vestibular areas; c/ Deciduous dentition. Dental malocclusion with anterior crossbite in the incisor region
Fig. 4
Fig. 4
PWS patients present thickened, sticky saliva. See the clinical manifestations in the images
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

References

    1. Belengeanu D, Bratu C, Stoian M, Motoc A, Ormerod E, Codruta Podariu U, et al. The heterogeneity of craniofacial morphology in Prader-Willi patients. Romanian J Morphol Embryol. 2012;53(3):527–532. - PubMed
    1. Skokauskas N, Sweeny E. Mental health problems in children with Prader-Willi syndrome. Acad Child Adolesc Psychiatry [Internet] 2012;21(3):194–203. - PMC - PubMed
    1. Cataletto M, Angulo M, Hertz G, Whitman B. Prader-Willi syndrome: A primer for clinicians. Int J Pediatr Endocrinol [Internet] 2011;2011(1):12. - PMC - PubMed
    1. Elena G, Bruna C, Benedetta M, Stefania DC, Giuseppe C. Prader-Willi syndrome: clinical aspects. J Obes. 2012;2012:1–12. - PMC - PubMed
    1. Bar C, Diene G, Molinas C, Bieth E, Casper C, Tauber M. Early diagnosis and care is achieved but should be improved in infants with Prader-Willi syndrome. Orphanet J Rare Dis. 2017;12(1):1–6. - PMC - PubMed