Original ArticlesDental abnormalities in patients with familial hypophosphatemic vitamin D-resistant rickets: Prevention by early treatment with 1-hydroxyvitamin D☆
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.
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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]