Conflict of interest: none.
X-linked hypophosphatemic rickets: Enamel abnormalities and oral clinical findings
Version of Record online: 27 MAR 2014
© 2014 Wiley Periodicals, Inc.
Volume 36, Issue 4, pages 456–461, July/August 2014
How to Cite
Cremonesi, I., Nucci, C., D'Alessandro, G., Alkhamis, N., Marchionni, S. and Piana, G. (2014), X-linked hypophosphatemic rickets: Enamel abnormalities and oral clinical findings. Scanning, 36: 456–461. doi: 10.1002/sca.21141
Author contribution: Ilaria Cremonesi, conceptualized, designed, acquisition of data and statistical analysis. Cesare Nucci, interpretation of the data, critical revision, and approval of the article. Giovanni D'Alessandro, data analysis, critical revision of the article. Nadia Alkhamis, conceptualized, designed, acquisition of data, and statistical analysis. Marchionni Silvia, SEM images. Gabriela Piana, interpretation of the data, critical revision, and approval of the article.
- Issue online: 7 AUG 2014
- Version of Record online: 27 MAR 2014
- Manuscript Accepted: 8 FEB 2014
- Manuscript Received: 4 DEC 2013
- University of Bologna, Department of Oral Science, Special Needs Patients Division (Pediatric Department in Sant'Orsola Hospital, Bologna, Italy)
- enamel abnormalities;
- large pulp chamber;
- scanning electron microscope;
- spontaneous abscesses;
- X-linked hypophosphatemic rickets (XLH)
X-linked hypophosphatemia (XLH) is a genetic disorder related to alterations in bones and teeth formation, due to low levels of phosphate in blood. Oral findings in XLH have been enamel and dentine abnormalities, high pulp horns, large pulp chambers, and some cases of periapical abscesses related to teeth without caries or traumatic injuries. The aim of our study was to assess the presence of enamel alterations, such as microclefts and/or structure defects in patients with XLH and give guidelines of prevention of XLH dental complications. History taking, oral clinical and radiological examination in 10 young patients affected by XLH (average age of 9) and in 6 patients without XLH (average age of 8). Impressions were performed on the vestibular surfaces of teeth in order to obtain replicas. The replicas were analyzed using scanning electron microscope (SEM) and compared to replicas of control group. The images of replicas of XLH patients showed deep microclefts and irregular enamel surface structure compared to replicas of control group. The replica of a patient with spontaneous periapical abscesses showed numerous enamel crater-shaped depressions and deep microcleavages penetrating into the enamel thickness. In absence of caries or fractures, the abscesses pathogenesis may be related to microcleavages of the enamel and dentin, which allow bacterial invasion of the pulp. There could be a relationship between XLH disease and enamel abnormalities. SCANNING 36:456–461, 2014. © 2014 Wiley Periodicals, Inc.