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
- 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
- Hennekam LR, Krantz ID, Allansonb IJ. Gorlin's Syndromes of the Head and Neck. Oxford University Press, Oxford, 2010
- DeLuke DM, Haug RH. Syndromes of the Head and Neck. Elsevier, Philadelphia, 2014
- Priolo M. Ectodermal dysplasias: An overview and update of clinical and molecular-functional mechanisms. American Journal of Medical Genetics, 149A:2003–2013, 2009
- 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
- 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
- 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
- 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
- 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
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