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Home Tooth developmental anomalies in severe combined immunodeficiency disease and juvenile myelomonocytic...

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

Authors:

  • G. Cossellu
    Department of Orthodontics, University of Milan, Orthodontic Department, Fondazione IRCCS C Granda, Ospedale Maggiore Policlinico, Milan, Italy
  • R. Seramondi
    Department of Orthodontics, University of Milan, Orthodontic Department, Fondazione IRCCS C Granda, Ospedale Maggiore Policlinico, Milan, Italy
  • S. Benedicenti
    University of Genoa, Department of Medical Science, Dentistry, and Biophysics, University of Genoa, Italy
  • G. Farronato
    School of Orthodontics, Department of Surgical, Reconstructive and Diagnostic Sciences, Fondazione IRCSS C Granda, University of Milan, Italy
  • G. Olivi
    Department of Endodontics, University of Genoa, Italy
  • F. Angiero
    University of Genoa, Department of Medical Science, Dentistry, and Biophysics, University of Genoa, Italy

ABSTRACT


Aim

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.

PLUMX METRICS

Publication date:

Dec /2013

Keywords:

juvenile myelomonocytic leukemia, severe combined immunodeficiency, tooth agenesis, tooth anomalies

Issue:

Vol.14 – n.4/2013

Page:

328 – 332

Publisher:

Ariesdue

Cite:


Harvard: G. Cossellu, R. Seramondi, S. Benedicenti, G. Farronato, G. Olivi, F. Angiero (2013) "Tooth developmental anomalies in severe combined immunodeficiency disease and juvenile myelomonocytic leukemia: common clinical features and treatment outcomes", European Journal of Paediatric Dentistry, 14(4), pp328-332. doi:
Vancouver: G. Cossellu, R. Seramondi, S. Benedicenti, G. Farronato, G. Olivi, F. Angiero. Tooth developmental anomalies in severe combined immunodeficiency disease and juvenile myelomonocytic leukemia: common clinical features and treatment outcomes. European Journal of Paediatric Dentistry [Internet]. 2013Dec.1 [cited 2023May.31];14(4):328-332. Available from: https://www.ejpd.eu/abstract-pubmed/tooth-developmental-anomalies-in-severe-combined-immunodeficiency-disease-and-juvenile-myelomonocytic-leukemia-common-clinical-features-and-treatment-outcomes/
MLA: G. Cossellu, R. Seramondi, S. Benedicenti, G. Farronato, G. Olivi, F. Angiero Tooth developmental anomalies in severe combined immunodeficiency disease and juvenile myelomonocytic leukemia: common clinical features and treatment outcomes. European Journal of Paediatric Dentistry. 2013;14(4):328-332

Copyright (c) 2021 Ariesdue

Creative Commons License

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

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    Editor in chief: dott. Luigi Paglia
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    European Journal of Paediatric Dentistry © | ISSN (Online): 2035-648X
    Registrazione del Tribunale di Milano n. 285 del 14.04.1998 | ROC 1946 - 26.09.2001
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