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Tooth developmental anomalies in severe combined immunodeficiency disease and juvenile myelomonocytic leukemia: common clinical features and treatment outcomes
 

Type:  Articles

Pubblication date:  12/2013

Authors:  G. Cossellu*, R. Seramondi*, S. Benedicenti**, G. Farronato***, G. Olivi****, F. Angiero**

Language:  English

Institution:  *Department of Orthodontics, University of Milan, Orthodontic Department, Fondazione IRCCS C Granda, Ospedale Maggiore Policlinico, Milan, Italy **University of Genoa, Department of Medical Science, Dentistry, and Biophysics, University of Genoa, Italy ***School of Orthodontics, Department of Surgical, Reconstructive and Diagnostic Sciences, Fondazione IRCSS C Granda, University of Milan, Italy ****Department of Endodontics, University of Genoa, Italy

Publication:  European Journal of Paediatic Dentistry

Publisher:  Ariesdue Srl

Keywords:  Juvenile Myelomonocytic Leukemia; Severe Combined Immunodeficiency; Tooth agenesis; Tooth anomalies.

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Email:  francesca.angiero@unige.it


Title:  Tooth developmental anomalies in severe combined immunodeficiency disease and juvenile myelomonocytic leukemia: common clinical features and treatment outcomes

Abstract:  Background Human Severe Combined Immunodeficiency (SCID) is a prenatal disorder of T lymphocyte development that depends on the expression of numerous genes. Juvenile myelomonocytic leukemia (JMML), previously known as juvenile chronic myeloid leukemia (JCML), is a rare, myelodysplastic/myeloproliferative disease typically presenting in early childhood. Case Reports Two cases are described of immunodeficiency disorders, both treated with chemotherapeutic drugs (Busulfan plus cyclophosphamide) before bone marrow transplantation. After treatment, these two different cases showed several similar oral lesions: microdontia, root alterations, numerous tooth ageneses, incomplete calcification, enamel hypoplasia, premature apexification and hypodontia. Both subjects underwent dental and orthodontic treatment. The first phase comprised orthopaedic treatment using a removable appliance (Interim-G) followed by rapid palatal expansion; in the second phase patients underwent tooth extraction and were treated using fixed appliances for 19 and 26 months, respectively (mean 2 years) to obtain final alignment and maximum intercuspation. In the third and final phase, reconstruction of malformed teeth was completed, and implant-supported protheses were applied. Conclusion The difficulties of managing and treating these diseases are discussed, with particular focus on tooth anomalies and malocclusion disorders. Collaboration between dentist and paediatrician in dealing with patients with a variety of oral lesions and tooth anomalies is important in order to prevent any other possible tooth lesions and ensure correct jaw development.

 
 
 
 
 
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