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1/5. Oral and dental manifestations of vitamin d-dependent rickets type I: report of a pediatric case.

    vitamin d-dependent rickets type I (VDDRI) represents an autosomal recessive hereditary defect in vitamin d metabolism. patients with VDDRI have mutations of chromosome 12 that affect the gene for the enzyme 1-alpha-hydroxylase, resulting in decreased levels of 1,25(OH)(2) vitamin d. Clinical features include growth failure, hypotonia, weakness, rachitic rosary, convulsions, tetany, open fontanels, and pathologic fractures. The oral and dental manifestations of VDDRI have not been described. Here we present the case of a 10-year-old girl affected by VDDRI, as established by the combination of clinical and radiographic findings, family history, and laboratory values. Dental examination revealed markedly hypoplastic, yellowish-to-brownish enamel in all permanent teeth, malocclusion, and chronic periodontal disease. Large quadrangular pulp chambers and short roots were evident in dental radiographs. light microscopic and ultrastructural examination showed abnormalities of dental hard tissues, affecting both enamel and dentin. The differential diagnosis and treatment of VDDRI are discussed.
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ranking = 1
keywords = rickets, vitamin
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2/5. Prophylactic dental treatment for a patient with vitamin d-resistant rickets: report of case.

    Spontaneous oral dental abscesses in caries-free teeth has been a common sequela in patients with vitamin d-resistant rickets (VDRR). A successful attempt has been made to prevent such abscesses in a 41/2-year-old boy with VDRR by covering susceptible teeth with chrome crowns.
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ranking = 0.99678597467111
keywords = rickets, vitamin
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3/5. vitamin d-dependent rickets type II: report of three cases.

    In this study the dental findings of three patients with vitamin d-dependent rickets (DDR) type II with alopecia, who were treated with large doses of vitamin D3 derivatives, are described. Dental development that is at or near a normal level can be achieved in controlled patients, permitting routine treatment of carious teeth.
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ranking = 0.98714389868444
keywords = rickets, vitamin
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4/5. Oral findings in digeorge syndrome.

    An 8-year-old female with digeorge syndrome was referred for dental treatment. Previous medical examination had disclosed heart and aortic arch malformations, hypoparathyroidism and an impaired cellular immune response. At dental examination, hypertelorism, a short philtrum, low-set malformed ears, a cleft palate and severe enamel hypoplasia were noted. The chronological distribution of enamel defects corresponded to the patients' age at known episodes of profound hypocalcemia occurring during treatment with vitamin d. Branchial dysembryogenesis should be considered in any case with dental changes related to hypoparathyroidism.
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ranking = 0.0032140253288889
keywords = vitamin
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5/5. The oral manifestations of intestinal lymphangiectasia: case report.

    Intestinal lymphangiectasia is a rare autosomal dominant disorder or acquired condition that leads to lymph obstruction, poor chyle transport, and concomitant problems of hypoproteinemia, lymphocytopenia, hypogammaglobulinemia, and peripheral edema. patients develop diarrhea, steatorrhea, and hypocalcemia secondary to fat-soluble vitamin malabsorption. Treatment is a restrictive diet of low fat, medium chain triglycerides. oral manifestations are gingivitis due to poor PMN function and enamel defects due to poor calcium absorption. A case of a 14-year-old boy with both gingival and enamel problems secondary to intestinal lymphangiectasia is reported.
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ranking = 0.0032140253288889
keywords = vitamin
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