Literature DB >> 19863895

Case series: Treatment considerations in x-linked hypohidrotic ectodermal dysplasia.

M O Lexner1, L Almer.   

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.

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Year:  2009        PMID: 19863895     DOI: 10.1007/bf03262696

Source DB:  PubMed          Journal:  Eur Arch Paediatr Dent        ISSN: 1818-6300


  21 in total

1.  Ectodermal dysplasias: how many?

Authors:  N Freire-Maia; T Lisboa-Costa; N A Pagnan
Journal:  Am J Med Genet       Date:  2001-11-15

2.  Children with ectodermal dysplasia need early treatment.

Authors:  Birgitta Bergendal
Journal:  Spec Care Dentist       Date:  2002 Nov-Dec

3.  X-linked anhidrotic (hypohidrotic) ectodermal dysplasia is caused by mutation in a novel transmembrane protein.

Authors:  J Kere; A K Srivastava; O Montonen; J Zonana; N Thomas; B Ferguson; F Munoz; D Morgan; A Clarke; P Baybayan; E Y Chen; S Ezer; U Saarialho-Kere; A de la Chapelle; D Schlessinger
Journal:  Nat Genet       Date:  1996-08       Impact factor: 38.330

4.  Christ-Siemens-Touraine syndrome--a clinical and genetic analysis of a large Brazilian kindred: III. Carrier detection.

Authors:  M Pinheiro; N Freire-Maia
Journal:  Am J Med Genet       Date:  1979

Review 5.  Hypohidrotic ectodermal dysplasia: characteristics and treatment.

Authors:  A Kupietzky; M Houpt
Journal:  Quintessence Int       Date:  1995-04       Impact factor: 1.677

6.  Anomalies of tooth formation in hypohidrotic ectodermal dysplasia.

Authors:  Michala O Lexner; Allan Bardow; Jens Michael Hertz; Lis A Nielsen; Sven Kreiborg
Journal:  Int J Paediatr Dent       Date:  2007-01       Impact factor: 3.455

7.  On the distribution of frequencies of mutation to genes determining harmful traits in man.

Authors:  A C Stevenson; C B Kerr
Journal:  Mutat Res       Date:  1967 May-Jun       Impact factor: 2.433

8.  The management of ectodermal dysplasia and severe hypodontia. International conference statements.

Authors:  J A Hobkirk; F Nohl; B Bergendal; K Storhaug; M K Richter
Journal:  J Oral Rehabil       Date:  2006-09       Impact factor: 3.837

9.  Expression of X-linked hypohidrotic ectodermal dysplasia in six males and in their mothers.

Authors:  A L Söderholm; I Kaitila
Journal:  Clin Genet       Date:  1985-08       Impact factor: 4.438

10.  Prosthetic management of hypohydrotic ectodermal dysplasia with anodontia. Case report.

Authors:  R M Shaw
Journal:  Aust Dent J       Date:  1990-04       Impact factor: 2.291

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  1 in total

Review 1.  Orthodontic and dentofacial orthopedic treatments in patients with ectodermal dysplasia: a systematic review.

Authors:  Marina Cerezo-Cayuelas; Amparo Pérez-Silva; Clara Serna-Muñoz; Ascensión Vicente; Yolanda Martínez-Beneyto; Inmaculada Cabello-Malagón; Antonio José Ortiz-Ruiz
Journal:  Orphanet J Rare Dis       Date:  2022-10-17       Impact factor: 4.303

  1 in total

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