Submitted Successfully!
Thank you for your contribution! You can also upload a video entry or images related to this topic.
Check Note
2000/2000
Ver. Summary Created by Modification Content Size Created at Operation
1 - + 186 word(s) 186 2020-09-09 08:03:28 |
2 format change -37 word(s) 149 2020-09-23 04:21:54 |
Ehlers-Danlos Syndromes
Edit
Upload a video

Ehlers–Danlos syndromes (EDS) are a clinically and genetically heterogeneous group of hereditary connective tissue disorders involving joint hyperlaxity, cutaneous hyperelasticity, and tissue fragility.

Ehlers Danlos Rare Diseases oral health
Information
Subjects: Pathology
Contributor MDPI registered users' name will be linked to their SciProfiles pages. To register with us, please refer to https://encyclopedia.pub/register :
View Times: 257
Revisions: 2 times (View History)
Update Date: 23 Sep 2020
Table of Contents

    1. Introduction

    A revised classification containing 13 subtypes was published by the International EDS Consortium, with a number of clinical conditions to guide and improve the diagnosis of each subtype[1]. In the European Union, a disease is considered ‘rare’ if it affects fewer than one in two thousand people[2]. In the case of EDS, the prevalence varies, depending on the type: between 1:30,000 (classical type) and < 1:1,000,000 (arthrochalasia type), whereas for the rarest form (the periodontitis type), the prevalence is unknown[3].

    EDS’ oral conditions have been described as periodontitis[1][4][5], temporomandibular dysfunctions[1][6], bleeding tendencies[7][8], enamel hypoplasia[1][9], shape abnormalities of the teeth and changes in the number of teeth[1][8], a high palate[1][10], dysgnathia, and malocclusion[1][11], as well as decreased effects of local anesthesia[12][13].

    References

    1. Fransiska Malfait; Clair Francomano; Peter Byers; John Belmont; Britta Berglund; James Black; Lara Bloom; Jessica M. Bowen; Angela F. Brady; Nigel P. Burrows; et al. The 2017 international classification of the Ehlers-Danlos syndromes.. American Journal of Medical Genetics Part C: Seminars in Medical Genetics 2017, 175, 8-26, 10.1002/ajmg.c.31552.
    2. Regulation (EC) No 141/2000 of the European Parliament and of the Council of 16 December 1999 on OrphanMedical Products. Available online: http://ec.europa.eu/health/files/eudralex/vol-1/reg_2000_141_cons-2009-07/reg_2000_141_cons-2009-07_en.pdf (accessed on 9 February 2018)
    3. The Portal for Rare Diseases and Orphan Drugs. Available online: http://www.orpha.net (accessed on 5 May 2019).
    4. Kapferer-Seebacher, I.; Lundberg, P.; Malfait, F.; Zschocke, J. Periodontal manifestations of Ehlers-Danlossyndromes: A systematic review.J. Clin. Periodontol.2017,44, 1088–1100.
    5. Medicina2020,56, 4488 of 95.Hanisch, M.; Hoffmann, T.; Bohner, L.; Hanisch, L.; Benz, K.; Kleinheinz, J.; Jackowski, J. Rare diseases withperiodontal manifestations.Int. J. Environ. Res. Public Health2019,16, 5.
    6. Peter J. Coster; Luc Martens; Anne Paepe; Oral health in prevalent types of Ehlers-Danlos syndromes. Journal of Oral Pathology & Medicine 2005, 34, 298-307, 10.1111/j.1600-0714.2004.00300.x.
    7. Létourneau, Y.; Pérusse, R.; Buithieu, H. Oral manifestations of Ehlers-Danlos syndrome.J. Can. Dent. Assoc.2001,67, 330–334.
    8. Abel, M.D.; Carrasco, L.R. Ehlers-Danlos syndrome: Classifications, oral manifestations, and dentalconsiderations.Oral Surg. Oral Med. Oral Pathol. Oral Radiol. Endod.2006,102, 582–590.
    9. Daniel H. Cohn; Peter H. Byers; Clinical Screening for Collagen Defects in Connective Tissue Diseases. Clinics in Perinatology 1990, 17, 793-809, 10.1016/s0095-5108(18)30546-3.
    10. Fridrich, K.L.; Fridrich, H.H.; Kempf, K.K.; Moline, D.O. D; ental implications in Ehlers-Danlos syndrome:A case report. Oral Surg. Oral Med. Oral Pathol. 1990, 69, 431–435, .
    11. Kevin A. Winters; Zhijie Jiang; Weihong Xu; Shibo Li; Zineb Ammous; Parul Jayakar; Klaas J. Wierenga; Re-assigned diagnosis of D4ST1-deficient Ehlers-Danlos syndrome (adducted thumb-clubfoot syndrome) after initial diagnosis of Marden-Walker syndrome. American Journal of Medical Genetics Part A 2012, 158, 2935-2940, 10.1002/ajmg.a.35613.
    12. Arendt-Nielsen, L.; Kaalund, S.; Bjerring, P.; Høgsaa, B. Insufficient effect of local analgesics in Ehlers Danlostype III patients (connective tissue disorder).Acta Anaesthesiol. Scand.1990,34, 358–361.
    13. Berglund, B.; Nordström, G.; Lützén, K. Living a restricted life with Ehlers-Danlos syndrome (EDS).Int. J.Nurs. Stud.2000,37, 111–118.
    More
    Information
    Subjects: Pathology
    Contributor MDPI registered users' name will be linked to their SciProfiles pages. To register with us, please refer to https://encyclopedia.pub/register :
    View Times: 257
    Revisions: 2 times (View History)
    Update Date: 23 Sep 2020
    Table of Contents
      1000/1000

      Confirm

      Are you sure to Delete?

      Video Upload Options

      Do you have a full video?
      Cite
      If you have any further questions, please contact Encyclopedia Editorial Office.
      Hanisch, M. Ehlers-Danlos Syndromes. Encyclopedia. Available online: https://encyclopedia.pub/entry/2113 (accessed on 08 June 2023).
      Hanisch M. Ehlers-Danlos Syndromes. Encyclopedia. Available at: https://encyclopedia.pub/entry/2113. Accessed June 08, 2023.
      Hanisch, Marcel. "Ehlers-Danlos Syndromes" Encyclopedia, https://encyclopedia.pub/entry/2113 (accessed June 08, 2023).
      Hanisch, M. (2020, September 22). Ehlers-Danlos Syndromes. In Encyclopedia. https://encyclopedia.pub/entry/2113
      Hanisch, Marcel. "Ehlers-Danlos Syndromes." Encyclopedia. Web. 22 September, 2020.
      Top