Witkop sendromu

Witkop sendromu (diş-tırnak sendromu; ectodermal dysplasia 3), otosomal dominant geçen kalıtsal bir sendromdur. Ektodermal displazi bulguları görece hafiftir.[1][2][3][4]

Dişlerle ilgili bulgular: Mikrodonti, taurodontism, hipodonti ve sürme (dentisyon) aksamaları başlıca bulgulardır.[2][3][5][6][7]

Tırnaklar ve deri: Saçlar incedir. Tırnaklarda yapı ve biçim bozuklukları saptanır.[1][2][3]

Tedavi

Tüm sendromlarda olduğu gibi, Witkop sendromu'nun da tedavisi yoktur. Tek amaç, hastaların yaşam niteliğini yükseltmektir. Witkop sendromu'nda dişlerle ilgili sorunların giderilmesi çabaları öne çıkar.[8][9]

Kaynakça
  1. Visinoni AF, Lisboa-Costa T, Pagnan NAB, Chautard-Freire-Maia EA. Ectodermal dysplasias: Clinical and molecular review. American Journal of Medical Genetics, 149A:1980–2002, 2009
  2. Hennekam LR, Krantz ID, Allansonb IJ. Gorlin's Syndromes of the Head and Neck. Oxford University Press, Oxford, 2010
  3. DeLuke DM, Haug RH. Syndromes of the Head and Neck. Elsevier, Philadelphia, 2014
  4. Priolo M. Ectodermal dysplasias: An overview and update of clinical and molecular-functional mechanisms. American Journal of Medical Genetics, 149A:2003–2013, 2009
  5. Wicomb GM, Stephen LX, Beighton P. Dental implications of Tooth-Nail dysplasia (Witkop syndrome): a report of an affected family and an approach to dental management. Journal of Clinical Pediatric Dentistry, 28(2):107-112, 2004
  6. Devadas S, Varma B, Mungara J, et al. Witkop tooth and nail syndrome: a case report. International Journal of Paediatric Dentistry, 15(5):364-369, 2005
  7. Ceyhan D, Kırzıoğlu Z, Calapoğlu NS. Mutations in the MSX1 gene in Turkish children with non-syndromic tooth agenesis and other dental anomalies. Indian Journal of Dentistry, 5(4):172-182, 2014
  8. Subramaniam P, Neeraja G. Witkop's tooth and nail syndrome: a multifaceted approach to dental management. Journal of Indian Society of Pedodontics and Preventive Dentistry, 26(1):22-25, 2008
  9. Kramer FJ, Baethge C, Tschernitschek H. Implants in children with ectodermal dysplasia: a case report and literature review. Clinical Oral Implants Research, 18:140–146, 2007
This article is issued from Wikipedia. The text is licensed under Creative Commons - Attribution - Sharealike. Additional terms may apply for the media files.