Gingival fibromatosis, short stature, border-line IQ, facial dysmorphism and hepatomegaly


Marakoglu I., Percin E., Gursoy U., Onarlioglu B., Ergur A.

GENETIC COUNSELING, cilt.16, ss.161-165, 2005 (SCI İndekslerine Giren Dergi) identifier identifier identifier

  • Cilt numarası: 16 Konu: 2
  • Basım Tarihi: 2005
  • Dergi Adı: GENETIC COUNSELING
  • Sayfa Sayıları: ss.161-165

Özet

Gingival fibromatosis, short stature, border-line IQ. facial dysmorphism and hepatomegaly. Gingival fibromatosis is a rare and benign disorder. The enlarged gingivae are firm and may Interfere with speech, closure of the lips, and mastication. We report a thirteen years old girl, with gingival fibromatosis referred to the periodontics clinics. Full mouth gingivectomy and gingivoplasty were performed. Medical investigation showed short stature, low-borderline IQ, facial dysmorphism. and hepatomegaly. Histological analysis revealed hyperplasia In the epithelial area and fibrotic appearance of gingival connective tissue with dense collagen fibre clusters. Pedigree analysis confirmed that mode of inheritance is autosomal recessive. According to the combination of clinical findings, this case report may represent a previously unreported syndrome.