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  • Title: Oral manifestations of familial hypophosphatemic rickets after phosphate supplement therapy: a review of the literature and report of case.
    Author: Larmas M, Hietala EL, Similä S, Pajari U.
    Journal: ASDC J Dent Child; 1991; 58(4):328-34. PubMed ID: 1658098.
    Abstract:
    The patient was a seven-year-old boy with familial vitamin D-resistant hypophosphatemic rickets. His mother, and her mother, were also affected. Before phosphate treatment was introduced in the patient, an impaired incorporation of calcium, and its exchange with sodium, was thought to be the principal etiological factor in the formation of globules. Supplementation therapy then resulted in a less elevated Ca/P ratio in the root area of the affected teeth, as well as a cure for the boy's bone structure. What the therapy did not cure was the globular appearance of the dentin and the hypomineralized stripe of pulpal horn extending to the cusp tips, an apparent permanent outcome of the disease.
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