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Home Ectodermal dysplasia: important role of complex dental care in its interdisciplinary...

Ectodermal dysplasia: important role of complex dental care in its interdisciplinary management

Authors:

  • L. Kratochvilova
    Department of Stomatology, Charles University, 2nd Medical Faculty and Motol University Hospital, Prague, Czech Republic
  • T. Dostalova
    Department of Stomatology, Charles University, 2nd Medical Faculty and Motol University Hospital, Prague, Czech Republic
  • M. Schwarz
    Department of Stomatology, Charles University, 2nd Medical Faculty and Motol University Hospital, Prague, Czech Republic
  • M. Macek Jr.
    Department of Biology and Medical Genetics, Charles University, 2nd Medical Faculty and Motol University Hospital, Prague, Czech Republic
  • I. Marek
    Institute of Dentistry and Oral Sciences, Palacky University, Faculty of Medicine and Dentistry and Faculty Hospital in Olomouc, Olomouc, Czech Republic
  • M. Malíková
    Department of Biology and Medical Genetics, Charles University, 2nd Medical Faculty and Motol University Hospital, Prague, Czech Republic
  • E. Misova
    Institute of Dentistry and Oral Sciences, Palacky University, Faculty of Medicine and Dentistry and Faculty Hospital in Olomouc, Olomouc, Czech Republic

DOI:

https://doi.org/10.23804/ejpd.2022.23.02.12

ABSTRACT


Aim

Despite the fact that ectodermal dysplasia (ED) is a rare disease, it is often seen in a tertiary clinic. ED affects ectodermal tissues such as skin, hair, teeth, nails, and sweat glands. Patients usually have sparse light hair, deformed nails, and dry skin. They suffer from dental abnormalities such as oligodontia (absence of 6 or more teeth) or complete anodontia; salivation can also be affected. The absence of teeth can be the overriding problem for both patients and their parents, and lead to substantial social ostracisation. This study aims to summarise the facts about the disease, especially dental treatment options based on data drawn from a representative Czech cohort.

Material and methods

The present article summarises the facts about ectodermal dysplasia (ED) in a cohort of 13 patients, where the following were evaluated: clinical manifestations of ED, pathogenic variants detected in selected candidate genes and dental treatment options from child removable dentures to fixed crowns and implants insertion. Three cases are described in detail and demonstrate approaches for different age groups.

Results

Our study highlights the need for awareness of the early signs of ED in dental and medical genetic practice. The ideal dental treatment plan includes interim removable dentures at a young age, orthodontic treatment in children and adolescents, and fixed restoration, including implants at a later date. With correct dental treatment, normal development of the patient’s jaw, as well as their self-esteem, is markedly improved.

Conclusion

Early diagnosis and active cooperation between the geneticist and dentist will facilitate cooperation with parents and patients and assure secondary prevention. It is preferable that the geneticist understands dental treatment options and can discuss these with patients/parents.

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Publication date:

June /2022

Keywords:

Dental abnormalities, dental treatment, ectodermal dysplasia

Issue:

Vol.23 – n.2/2022

Page:

140 – 146

Publisher:

Ariesdue

Topic:

Children with special health care needs

Cite:


Harvard: L. Kratochvilova, T. Dostalova, M. Schwarz, M. Macek Jr., I. Marek, M. Malíková, E. Misova (2022) "Ectodermal dysplasia: important role of complex dental care in its interdisciplinary management", European Journal of Paediatric Dentistry, 23(2), pp140-146. doi: 10.23804/ejpd.2022.23.02.12
Vancouver: L. Kratochvilova, T. Dostalova, M. Schwarz, M. Macek Jr., I. Marek, M. Malíková, E. Misova. Ectodermal dysplasia: important role of complex dental care in its interdisciplinary management. European Journal of Paediatric Dentistry [Internet]. 2022Jun.17 [cited 2023Feb.03];23(2):140-146. Available from: https://www.ejpd.eu/abstract-pubmed/ectodermal-dysplasia-important-role-of-complex-dental-care-in-its-interdisciplinary-management/
MLA: L. Kratochvilova, T. Dostalova, M. Schwarz, M. Macek Jr., I. Marek, M. Malíková, E. Misova Ectodermal dysplasia: important role of complex dental care in its interdisciplinary management. European Journal of Paediatric Dentistry. 2022;23(2):140-146

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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    Cristina Calchera
    Editor in chief: dott. Luigi Paglia
    European Journal of Paediatric Dentistry © | ISSN (Online): 2035-648X
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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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