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European Archives of Paediatric Dentistry

, Volume 10, Supplement 1, pp 26–30 | Cite as

Treatment considerations in x-linked hypohidrotic ectodermal dysplasia

  • M. O. LexnerEmail author
  • L. Almer
Case series

Abstract

AIMS: To describe our treatment considerations obtained from the dental care of several boys affected with X-linked hypohidrotic ectodermal dysplasia (HED) and to discuss the different problems that may arise in connection with these kinds of treatments. STUDY DESIGN: The subject group included 10 males affected with X-linked HED, treated at the department of Paediatric Dentistry and Clinical Genetics, Copenhagen School of Dentistry. All patients were treated, after a period of adaptation, with removable prostheses and whenever necessary, composite restoration of the conical crown morphology of the maxillary incisors. Furthermore, some of the patients received an orthodontic treatment for closure/reduction of the medial diastema. RESULTS: The mean number of appointments at the department was 32 with a range from 7 to 59 appointments. In 7 out of 10 patients, the treatment was a success according to the patient, parents and dentist. In 3 out of 10 patients the treatment was not a success from the dentist’s point of view: primarily due to lack of cooperation between the patient, parents and dentist. In half of the patients the deviated maxillary incisors morphology was restored and 5 out of 10 patients received orthodontic treatment for closure of the medial diastema in the maxilla. CONCLUSION: It is of utmost importance to take the motivation of the patient and parents as well as the patient’s acceptance for dental treatment into consideration. Furthermore, the treatment is rather time-consuming and should involve different areas of specialisation. Finally it is vital to remember that the parents may also have undergone a prolonged dental treatment, which might reduce their patience regarding their children’ s treatments.

Key words

Hypohidrotic ectodermal dysplasia dental abnormalities dental treatments affected males 

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References

  1. Bayés M, Hartung AJ, Ezer S, et al. The anhidrotic ectodermal dysplasia gene (EDA) undergoes alternative splicing and encodes ectodysplasin-A with deletion mutations in collagenous repeats. Hum Mol Genet 1998; 7: 1661–9.PubMedCrossRefGoogle Scholar
  2. Boj JR, Von Arx JD, Cortada M, Jimenez A, Golobart J. Dentures for a 3-yr-old child with ectodermal dysplasia. Am J Dent 1993; 6: 165–7PubMedGoogle Scholar
  3. Bergendal B, Kurol J. Oral rehabilition of patients with EDA-case presentation. In Bergendal B, Koch G, Kurol J, Wänndahl G (eds.). Consensus conference on ectodermal dysplasia with special reference to dental treatment. Stockholm: Förlagshuset Gothia 1988: 77–99.Google Scholar
  4. Bergendal B. Children with ectodermal dysplasia need early treatment. Spec Care Dentist 2002; 22: 212–3.PubMedCrossRefGoogle Scholar
  5. Carter CO. Monogenic disorders. J Med Genet 1977; 14: 316–20.PubMedCrossRefGoogle Scholar
  6. Crawford PJM, Aldred MJ, Clarke A. Clinical and radiographic findings in X linked hypohidrotic ectodermal dysplasia. J Med Genet 1991; 28: 181–5.PubMedCrossRefGoogle Scholar
  7. Farrington FH. The team approach to the management of ectodermal dysplasia. In: Salinas CF, Opit JM, Paul NW (eds). Recent advances in ectodermal dysplasia. New York: Alan R. Liss 1988: 243–52.Google Scholar
  8. Freire-Maia N, Pinheiro M. Ectodermal dysplasia: A Clinical and Genetic Study, Alan R.Liss, New York, 1984: 22–7,33-6,183-5,204-5.Google Scholar
  9. Freire-Maia N, Lisboa-Costa T, Pagnan NAB. Ectodermal dysplasias. How many? Am J Med Genet 2001; 104: 84.PubMedCrossRefGoogle Scholar
  10. Gorlin RJ, Cohen MM, Hennekam RCM JR. Syndromes of the Head and Neck, Oxford University press, 2001: 540–1.Google Scholar
  11. Guckes AD, Brahim JS, Mccarthy GR, Rudy SF, Cooper LF. Using endosseous dental implants for patients with ectodermal dysplasia. J Am Dent Assoc 1991; 122: 59–62PubMedGoogle Scholar
  12. Hickey AJ, Vergo TJ. Prosthetic treatments for patients with ectodermal dysplasia. J Prosthet Dent 2001; 86: 364–8.PubMedCrossRefGoogle Scholar
  13. Hobkirk JA, Nohl F, Bergendal K, Storhaug K, Richter MK. The managment of ectodermal dysplasia and severe hypodontia. International conference statements. J Oral Rehab 2006; 33: 634–7.CrossRefGoogle Scholar
  14. Kere J, Srivastava AK, Montonen O, et al. X linked anhidrotic (hypohidrotic) ectodermal dysplasia is caused by mutation in a novel transmembrane protein. Nat Genet 1996; 13: 409–16.PubMedCrossRefGoogle Scholar
  15. Kupietzky A, Houpt M. Hypohidrotic ectodermal dysplasia: Characteristics and treatment. Quintessence Int 1995; 26: 285–91PubMedGoogle Scholar
  16. Lexner MO, Bardow A, Hertz JM, Nielsen LA, Kreiborg S. Anomalies of tooth formation in hypohidrotic ectodermal dysplasia. Int J Paediatr Dent 2007a; 17: 10–8.PubMedCrossRefGoogle Scholar
  17. Lexner MO, Bardow A, Hertz JM, et al. Whole saliva in X-linked hypohidrotic ectodermal dysplasia. Int J Paediatr Dent 2007b; 17: 155–62.PubMedCrossRefGoogle Scholar
  18. Mikkola ML, Pispa J, Pekkanen M, et al. Ectodysplasin, a protein required for epithelial morphogenesis, is a novel TNF homologue and promotes cellmatrix adhesion. Mech Dev 1999; 88: 133–46.PubMedCrossRefGoogle Scholar
  19. Nordgarden H, Storhaug K, Lyngstadaas SP, Jensen JL. Salivary glands function in persons with ectodermal dysplasia. Eur J Oral Sci 2003; 111: 3716.CrossRefGoogle Scholar
  20. Nowak AJ. Dental treatment of patients with ectodermal dysplasia. In: Salinas CF, Opitz JM, Paul NW (eds). Recent advances in ectodermal dysplasia. New York: Alan R. Liss 1988: 243–52.Google Scholar
  21. Nunn JH, Carter NE, Gillgrass TJ, et al. The interdisciplinary management of hypodontia: background and role of paediatric dentistry. Brit Dent J 2003; 194: 245–51.PubMedCrossRefGoogle Scholar
  22. Pinheiro M, Freire-Maia N. Christ-Siemens-Touraine syndrom — a clinical and genetic analysis of a large brazilian kindred: III. Carrier detection. Am J Med Genet 1979; 4: 129–34.PubMedCrossRefGoogle Scholar
  23. Shaw RM. Prosthetic management of hypohidrotic ectodermal dysplasia with anodontia. Case report. Aust Dent J 1990: 35: 113–6.PubMedCrossRefGoogle Scholar
  24. Snawder K. Considerations in dental treatment of children with ectodermal dysplasia. J Am Dent Assoc 1976; 93: 1177–9PubMedGoogle Scholar
  25. Stevenson AC, Kerr CB. On the distributions of frequencies of mutation to genes determining harmful traits in man. Mutat Res 1967; 4: 339–52.PubMedCrossRefGoogle Scholar
  26. Söderholm AL, Kaitila I. Expression of X-linked hypohidrotic ectodermal dysplasi in six males and in their mother. Clin Genet 1985; 28: 136–44.PubMedCrossRefGoogle Scholar

Copyright information

© Adis International 2009

Authors and Affiliations

  1. 1.Department of Paediatric Dentistry and Clinical Genetics, School of DentistryUniversity of CopenhagenCopenhagen NDenmark

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