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BECKWITH - WIEDEMANN SENDROMU (OLGU BİLDİRİSİ)

THE BECKWITH - WIEDEMANN SYNDROME (CASE REPORT)

Journal Name:

  • İstanbul Üniversitesi Diş Hekimliği Fakültesi Dergisi

Publication Year:

  • 1994

Key Words:

Keywords (Original Language):

Author NameUniversity of AuthorFaculty of Author
Abstract (2. Language): 
Beckwith-Wiedemann Syndrome is a growth disorder, with cardinal symptoms of exomphalos, macroglossia and gigantism. The etiology of the disorder is unknown. However, the review of the cases reported, shows that the syndrome is inherited by way of, an austosomal dominant gene with various expressivity. Some authors report its prevalance as 1/13.700. Females are more affected by 60%. The Syndrome is as important condition in dental practice as it has features like macroglossia, malocclusion with tendency toward mandibular prognathism. This article, is the report of a case of Beckwith-Wiedemann Syndrome which was diagnosed at birth. The case was previously operated for omphalocele and macroglossia; and later applied to our clinic for the treatment of dental caries.
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Abstract (Original Language): 
Beckwith-Wiedemann sendromu , ana belirtileri 'exomp-halos', makroglossi ve gigantizm olan ve etiyolojisi henüz tamamen aydınlanmamış bir büyüme hastalığıdır. Ancak bugüne kadar sendromun değişik expresivite gösteren oto-somal dominant bir genle geçtiğini bildiren vakalar vardır. Bazı yazarlar sendromun görülme sıklığım 1/13.700 olarak bildirmişlerdir. Kızlarda yaklaşık %60 oranında daha fazla görülmektedir. Diş Hekimliği açısından bu sendrom, makroglossi, mandi-buler prognatiye eğilim ile birlikte maiokfüzyonlann görülmesi nedeniyle önemlidir. Bu yazıda doğumda tanımlanmış 'omphalocele' ve mak-roglosi ameliyatı geçirmiş olan ve kliniğimize diş çürükleri sebebiyle başvuran bir Beckwith-Wiedemann olgusu sunulmuştur.
FULL TEXT (PDF): 
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  • 3
180
184
  • Turkish

REFERENCES

References: 

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2.
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