The care of the patient with X-linked hypophosphatemic rickets

Authors:

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.

Results

XLH patients showed enlarged pulp and altered morphology with prominent pulp horns into the tooth crown. Deficiency of 1,25-dihydroxyvitamin D induced by Fibroblast Growth Factor 23 resulted in enamel, dentin, and cement defects. Histological analysis demonstrated a poor mineralisation of these structures with significant irregularities. Patients with a history of abscesses were 67; canines and molars are less affected than incisors.

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.

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