Abstract

Aim: Familial Tumoral Calcinosis (FTC) is a rare autosomal recessive disorder characterized by ectopic calcifications in periarticular location. The hip, shoulder and elbow are common locations. The foot, leg, Knee and hand are involved less frequently. FTC can be due to mutations in GALNT3 gene encoding for glycosyltransferase, a protein that helps to regulate phosphate levels, or Fibroblast Growth Factor 23 gene (FGF 23). This is secreted by bone cells and acts at the kidney to regulate phosphate reabsorption and vitamin D3 production. The aim of our work is to describe a case of FTC with dental anomalies and bisphosphonates therapy undergone to orthodontic and surgical treatment. Materials and Metods: A 13 years-old boy with FTC was referred to our observation and he underwent a complete clinical examination and biochemical, radiological, genetic testing : to intraoral examination teeth are smaller than normal with enamel hypoplasia, panoramic radiographs revealed unerupted 1.3, 2.3, 3.3, 4.3 and tooth anomalies ( short bulbous teeth with obliteration of the pulp chamber and root canal), x-ray pelvis showed calcified areas around of both hip joints. Laboratory tests showed hyperphosphatemia, normocalcemia, elevated level of parathyroid hormone and normal level of 1,25 vitamin D3 and DNA sequencing identified a mutation in GALNT 3 gene. After cephalometric analysis of lateral teleradiographs of the skull an orthodontic treatment with rapid maxillary expansion and subsequent alignment was performed. Results: Orthodontic treatment has solved the malocclusion with the correction of skeletal relationship and dental class. Discussion: Considering the characteristics of the disease which has an alteration of bone metabolism, the assumption of biphosphates which reduce dental movement and the presence of teeth with anomolous roots in a short and stocky form, it has been thought convenient to proceed with an orthodontic treatment using light force (.012, .014, .016 N.T. wires) even though the application of the E.R.P. has given excellent results with the opening of the medial palatine suture.Conclusion: Literature doesn't describe cases of patients having FTC orhodontic and orhtopaedic treatment. Our experience shows that timely management of orthodontic forces allows reaching positive results which remain stable in time (2 year follow-up).

Autore Pugliese

• Favia Gianfranco , Lacaita Maria Grazia

Tutti gli autori

• FAVIA G.;LACAITA M.G.

Titolo volume/Rivista

Non Disponibile

Anno di pubblicazione

2010

ISSN

0026-4970

ISBN

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Numero di citazioni Wos

Nessuna citazione

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Numero di citazioni Scopus

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Ultimo Aggiornamento Citazioni

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Settori ERC

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Codici ASJC

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