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RIEGER SENDROMU' (OLİGODONTİ VE PRİMER MEZODERMAL İRİS DİSGENEZİSİ)

RIEGER'S SYNDROME (OLIGODONTIA AND PRIMARY MESODERMAL DYSGENESIS OF THE IRIS)

Journal Name:

  • İstanbul Üniversitesi Diş Hekimliği Fakültesi Dergisi

Publication Year:

  • 1993

Key Words:

Keywords (Original Language):

Author NameUniversity of AuthorFaculty of Author
Abstract (2. Language): 
Rieger's syndrome is a rare autosomal dominant disorder characterized by dental and ocular abnormalities. The essential ocular findings of the Rieger's syndrome are bilateral hypoplasia of the iris, iris adhesions, microcornea, sclerocornea, congenital opacities and glaucoma. The diagnosis of the Rieger's syndrome can be differentiated from other anterior chamber malformation syndromes by the presence of characteristic dental abnormalities. Oral anomalies associated with Rieger's syndrome are hypodontia in anterior maxillary segment, microdontia, misshaped teeth, delayed eruption of the teeth and conical crowns. Characteristic facial findings are hypoplasia of premaxilla and relative mandibular prognatism. Case reported in this article describes characteristics of the Rieger's syndrome
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Abstract (Original Language): 
Rieger Sendromu az rastlanan ve otozomal dominant kalıtımla geçen bir hastalıktır. En önemli bulgular göz ön kamerası defektleri ve dişlerle ilgili gelişim malformas-yonlarıdır. Göz ile ilgili bulgular bilateral iris hipoplazisi, doğumsal iris yapışması, mikrokornea, sklerokornea, strabismus, doğumsal opazite ve glokomdur. Göz ön kamerasını ilgilendiren diğer hastalıklardan dişse] anomalilerin varlığı ile ayırt edilir. Rieger sendromu ile ilgili oral anomaliler ön maksiller bölgede hipodonti, mikrodonti, normal olmayan şekillenme ve sürme gecikmeleridir. Hipop-lazik premaksilla ile relatif belirgin mandibuler prognali ise karakteristik yüz bulgularıdır. Bildirilen olgu tipik göz bulgularının karakteristik ağız ve yüz bulguları ile desteklendiği bir Rieger sendromu olgusudur.
FULL TEXT (PDF): 
PDF icon arastirmax-rieger-sendromu-oligodonti-primer-mezodermal-iris-disgenezisi.pdf
  • 3
193
197
  • Turkish

REFERENCES

References: 

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