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Clinical approach in familial hypophosphatemic rickets: report of three generations

Identifieur interne : 002D16 ( Main/Exploration ); précédent : 002D15; suivant : 002D17

Clinical approach in familial hypophosphatemic rickets: report of three generations

Auteurs : Eduardo Costa Studart Soares [Brésil] ; Fábio Wildson Gurgel Costa [Brésil] ; Thyciana Rodrigues Ribeiro [Brésil] ; Ana Paula Negreiros Nunes Alves [Brésil] ; Cristiane Sá Roriz Fonteles [Brésil]

Source :

RBID : ISTEX:FFB6EB12E25DD3B4E04618C254B9D11D9CB4DCE7

English descriptors

Abstract

Familial hypophosphatemic rickets is a hereditary disease characterized by the involvement of several family members, transmitted in most cases as an X‐linked dominant trait. Oral manifestations can be the first evidences for an adequate and early diagnosis of X‐linked hypophosphatemic rickets (XLHR). The present report describes the main systemic manifestations, oral findings and dental management in three generations of an affected family. Oral exams, laboratorial and histologic evaluations, cone‐beam computed tomographies, panoramic and periapical radiographs were performed to properly institute the most adequate treatment strategy. The knowledge of clinical signs and symptoms of XLHR is essential for the correct diagnosis of this disease, and for the establishment of preventive and comprehensive dental care.

Url:
DOI: 10.1111/j.1754-4505.2012.00310.x


Affiliations:


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<name sortKey="Costa, Fabio Wildson Gurgel" sort="Costa, Fabio Wildson Gurgel" uniqKey="Costa F" first="Fábio Wildson Gurgel" last="Costa">Fábio Wildson Gurgel Costa</name>
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<name sortKey="Ribeiro, Thyciana Rodrigues" sort="Ribeiro, Thyciana Rodrigues" uniqKey="Ribeiro T" first="Thyciana Rodrigues" last="Ribeiro">Thyciana Rodrigues Ribeiro</name>
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<name sortKey="Alves, Ana Paula Negreiros Nunes" sort="Alves, Ana Paula Negreiros Nunes" uniqKey="Alves A" first="Ana Paula Negreiros Nunes" last="Alves">Ana Paula Negreiros Nunes Alves</name>
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<name sortKey="Fonteles, Cristiane Sa Roriz" sort="Fonteles, Cristiane Sa Roriz" uniqKey="Fonteles C" first="Cristiane Sá Roriz" last="Fonteles">Cristiane Sá Roriz Fonteles</name>
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<term>Adult patients</term>
<term>Arabic numerals</term>
<term>Black arrow</term>
<term>Bone loss</term>
<term>Cear</term>
<term>Clinical dentistry</term>
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<term>Deciduous dentition</term>
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<term>Early loss</term>
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<term>Familial hypophosphatemic rickets</term>
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<term>First evidences</term>
<term>Gingival</term>
<term>Histologic</term>
<term>Hypophosphatemic</term>
<term>Hypophosphatemic rickets</term>
<term>Initial presentation</term>
<term>Large pulp chambers</term>
<term>Many teeth</term>
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<term>Oral findings</term>
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<term>Pediatric patients</term>
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<term>Periodontal disease</term>
<term>Permanent dentitions</term>
<term>Phex gene</term>
<term>Present case report</term>
<term>Proband</term>
<term>Pulp horns</term>
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<term>Radiol endod</term>
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<term>Roman numerals</term>
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<term>Present case report</term>
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<div type="abstract">Familial hypophosphatemic rickets is a hereditary disease characterized by the involvement of several family members, transmitted in most cases as an X‐linked dominant trait. Oral manifestations can be the first evidences for an adequate and early diagnosis of X‐linked hypophosphatemic rickets (XLHR). The present report describes the main systemic manifestations, oral findings and dental management in three generations of an affected family. Oral exams, laboratorial and histologic evaluations, cone‐beam computed tomographies, panoramic and periapical radiographs were performed to properly institute the most adequate treatment strategy. The knowledge of clinical signs and symptoms of XLHR is essential for the correct diagnosis of this disease, and for the establishment of preventive and comprehensive dental care.</div>
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<name sortKey="Costa, Fabio Wildson Gurgel" sort="Costa, Fabio Wildson Gurgel" uniqKey="Costa F" first="Fábio Wildson Gurgel" last="Costa">Fábio Wildson Gurgel Costa</name>
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