TİRALİ, Resmiye Ebru Başkent Üniversitesi - Diş Hekimliği Fakültesi - Pedodonti Anabilim Dalı, Turkey , BODUR, Haluk Gazi Üniversitesi - Diş Hekimliği Fakültesi - Pedodonti Anabilim Dalı, Turkey

MUCOPOLYSACCHARIDOSIS TYPE-IVA ( MORQUIO S SYNDROME)

26144

Morquio’s Syndrome is a rare skeletal dysplasia with an autosomal recessive trait inheritance that has characterized with disorder of mucopolysaccharide metabolism. The deficiency of N-acetylgalactosamine-6-sulfate sulphatase cause TypeAform, and beta galactosidase deficiency cause Type B form. Dental defects are rarely seen in association with the syndrome. Afourteen year-old female withMorquio syndrome diagnosis was referred to the clinic of Gazi University Faculty of Dentistry Department of Paediatric Dentistry for function and esthetic problems. These problems was treated and consultated with ortodontics. Patient was followed up.

37

Morquio Syndrome , dental findings

Acta Odontologica Turcica

40