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Gardner sendromu (familial adenomatous polyposis 1)

Kalıtsal - AD

Familial adenomatöz polipozis sendromunun 3 fenotipinden biri (ötekiler: Brain tumor-polyposis syndrome 2, familial adenomatous polyposis)

Multipl osteomalar: çeneler, frontal, zigomatik, etmoid ve sfenoid kemiklerde

Deride erozyon (yüz, gövde, ekstremite)

Çene tümörleri: odontomalar, osteoid osteoma, benign osteoblastoma

Süpernümerer dişler

Mesiodens

Gömük dişler

Yaygın çürükler

Gözde retina kalınlaşması

Abdominal/ekstraabdominal desmoid oluşumlar

Fibromlar (Gardner fibromaları)

Mezenterde mezenkimal tümörler (lipoma, fibroma, fibrosarkoma)

Kalın bağırsak polipleri ve kanserleşmeleri

Adrenal tümörleri

Tiroid tümörleri

Beyin tümörleri

 

  • Chan MY, Shifteh K, Shanske AL. Mesiodens, a new microform of Holoprosencephaly? American Journal of Medical Genetics, 149A:268–271, 2009

  • Oliveira MR, Rodrigues WC, Gabrielli MF, et al. Gardner syndrome with unusual maxillofacial manifestation. Journal of Craniofacial Surgery, 27(5):1253-1255, 2016

  • Lubinsky M, Kantaputra PN. Syndromes with supernumerary teeth. American Journal of Medical Genetics, 170A:2611–2616, 2016

  • Koh KJ, Park HN, Kim KA. Gardner syndrome associated with multiple osteomas, intestinal polyposis, and epidermoid cysts. Imaging Science in Dentistry, 46(4):267-272, 2016

  • Li J, Woods SL, Healey S, et al. Point mutations in exon 1B of APC reveal gastric adenocarcinoma and proximal polyposis of the stomach as a familial adenomatous polyposis variant. American Journal of Human Genetics,98: 830-842, 2016

  • Seehra J, Patel S, Bryant C. Gardner's Syndrome revisited: a clinical case and overview of the literature. Journal of Orthodontics, 43(1):59-64, 2016

  • Argyris PP, Koutlas IG. Orthokeratinized odontogenic cyst with an associated keratocystic odontogenic tumor component and ghost cell keratinization and calcifications in a patient with Gardner Syndrome. Head & Neck Pathology, 11(2):240-248, 2017