Original Articles
Dental abnormalities in patients with familial hypophosphatemic vitamin D-resistant rickets: Prevention by early treatment with 1-hydroxyvitamin D

https://doi.org/10.1067/mpd.2003.119Get rights and content

Abstract

Objective To evaluate the dental effects of 1-hydroxylated vitamin D3 treatment in patients with familial hypophosphatemic vitamin D–resistant rickets. Study design Forty-eight children and adult patients were included in the study; 16 had received no treatment or phosphate supplements with vitamin D/25-(OH) D3 before puberty. The 32 younger ones had received phosphate supplements with 1α-(OH)D3 from infancy. All patients were clinically examined, and panoramic and periapical radiographs were made. Evaluations of decayed, missing, or filled teeth and decayed or filled teeth indexes and of pulp ratios allowed comparison with healthy age-matched control patients. Results Poor dental health and characteristic dental anomalies were found in the 16 older patients. In contrast, the 32 younger patients had a normal dental status as regards reference ranges in healthy age-matched populations, although they still showed prominent pulp horns on deciduous teeth and increased pulp area/tooth area ratios. Conclusions This investigation shows the beneficial effects of 1α-(OH)D3 treatment on the dental status of vitamin D–resistant rickets patients and emphasizes the necessity of early treatment. Remaining defects may result from early exposure of odontoblasts and surrounding osteoblasts to hypophosphatemia, before the commencement of treatment, and/or from intrinsic cell disturbances linked to the genetic alteration(s). (J Pediatr 2003;142:324-31)

Section snippets

Methods

We examined 48 children and adults with VDRR regularly followed as outpatients in the Saint Vincent de Paul Hospital, Paris, and analyzed the results as regards the vitamin D form administrated during childhood and their compliance to treatment. Patients were recruited over a period of 18 months on the occasion of a systematic outpatient follow-up; 39 had been followed since early childhood in our department (age, 3-35 years). They had received vitamin D or 1α-hydroxy vitamin D3 (Unalfa, Leo

Patients Treated With 1α-(OH)D3 From Infancy Had a Normal Dental History

The 16 hypophosphatemic patients born before 1970 had poor dental health (Table I).One patient had lost all his teeth and 8 others had 2 missing teeth or more, with a mean percentage of missing teeth per patient of 22%. All patients with some teeth had necrosed or endodontically treated teeth, and the mean percentage of pulpally diseased or treated teeth in this group was high (58%; range, 25-100%).

By contrast, only 2 of the patients born after 1970 had permanent missing teeth (1 tooth in a

Discussion

Familial hypophosphatemic vitamin D– resistant rickets is classically associated with several dental anomalies.8, 9, 10, 11, 12, 13, 14, 15, 16 These alterations lead to spontaneous dental abscesses and loss of primary and permanent teeth. Yet, the causal mechanism(s) remain unclear. Part of the mineralization defects may result from the abnormal environment of the dental and peridental cells, as patients with an altered PHEX gene have low concentration of phosphates in the extracellular fluids

Acknowledgements

We thank Professor Mel Grumbach, PhD (San Francisco,CA), and Pr Michel Goldberg, PhD (Paris, France), for reading the manuscript and for helpful discussion. We also thank Pr Denis Bourgeois, PhD (Lyon, France), for his advice on national data and indexes.

References (32)

  • R Guo et al.

    Cloning and sequencing of human PEX from a bone cDNA library: evidence for its developmental stage-specific regulation in osteoblasts

    J Bone Miner Res

    (1997)
  • AF Ruchon et al.

    Pex mRNA is localized in developing mouse osteoblasts and odontoblasts

    J Histochem Cytochem

    (1998)
  • T Nesbitt et al.

    Coordinated maturational regulation of PHEX and renal phosphate transport inhibitory activity: evidence for the pathophysiological role of PHEX in X-linked hypophosphatemia

    J Bone Miner Res

    (1999)
  • CJ Witkop

    Manifestations of genetic diseases in the human pulp

    Oral Surg Oral Med Oral Pathol

    (1971)
  • ME Pliskin et al.

    Vitamin resistant rickets of a young adult patient

    J Oral Med

    (1975)
  • EN Tulloch et al.

    The association of dental abscesses with vitamin D resistant rickets

    Br Dent J

    (1983)
  • Cited by (0)

    Reprint requests: Catherine Chaussain-Miller, MD, Dental School of University of Paris 5, Department of Conservative Dentistry and Endodontics, 1 rue Maurice Arnoux, 92120 Montrouge, France. E-mail: [email protected]

    View full text