The care of the patient with X-linked hypophosphatemic rickets

Authors

  • I Capotosti Department of Surgical, Medical, Molecular and Critical Area Pathology, Unit of Pediatric Dentistry, University of Pisa, Pisa
  • GI Baroncelli Pediatric and Adolescent Endocrinology, Division of Pediatrics, Department of Obstetrics, Gynecology and Pediatrics, University Hospital, Pisa
  • S Ligori Department of Surgical, Medical, Molecular and Critical Area Pathology, Unit of Pediatric Dentistry, University of Pisa, Pisa
  • E Zampollo Department of Surgical, Medical, Molecular and Critical Area Pathology, Unit of Pediatric Dentistry, University of Pisa, Pisa
  • MR Giuca Department of Surgical, Medical, Molecular and Critical Area Pathology, Unit of Pediatric Dentistry, University of Pisa, Pisa
  • E Carli Department of Surgical, Medical, Molecular and Critical Area Pathology, Unit of Pediatric Dentistry, University of Pisa, Pisa

DOI:

https://doi.org/10.23804/ejpd.2024.25.04.02

Keywords:

X-linked hypophosphatemia, periapical dental abscesses, PHEX gene, dentin enamel, periodontal disease

Abstract

AIM: X-linked hypophosphatemic rickets (XLH) is characterised by severe skeletal deformities and dental abnormalities. The aim of this work is to examine the prevalence of abscesses, the features of pulp chambers, and the biochemical and histological signs of the disease. METHODS: Pulp chambers size, shape, and morphology were assessed by orthopantomography in XLH patients (n = 24, age 5.8 ± 1.6 years) and in sex and age-matched healthy controls (n = 23, age 6.2 ± 1.4 years). Histological analysis of the dentin of the primary teeth by Hematoxylin-eosin and Massontrichromic Goldner-blue aniline staining was then performed, comparing the results with those obtained in healthy controls. CONCLUSION: Enlarged pulpal chambers with altered shape and morphology affect most XLH patients, setting them up for periapical abscesses with fistulas occurred without any history of trauma or dental decay. Patients with XLH should be considered high-risk patients. It is important to treat and manage them early by a multidisciplinary approach.