SOS1 ASSOCIATED NOONAN SYNDROME PRESENTING WITH SUPERNUMARY TEETH; A CASE REPORT
Makaleler > Tam Makale · 2015
Yazarlar: Gülser KILINIÇ, Özge AKSEL, Müjdat ÇETİN, Hüseyin ONAY, Özlem Giray BOZKAYA
Atatürk Üniversitesi Diş Hekimliği Fakültesi Dergisi, Uluslararası, 2015, cilt 12
DOI: 10.17567/dfd.61402
Özet
Noonan syndrome (NS) is a common autosomal dominant disorder which is characterized by chest deformation, congenital heart disease, short stature and distinctive facial features.Supernumerary teeth are rarely seen in connection with NS, however severe gingivitis could be seen in NS patients more frequently.In this report, a 12-year-old boy with Noonan syndrome whose oral examination revealed an anterior open bite, supernumerary teeth, severe anterior gingival enlargement, dental crowding associated with diestema, tapered incisors, narrow high-arched palate and prominent rugae was presented.Mutation analysis of the BRAF, PTPN11 and SOS1 genes from peripheral blood cells showed that our patient has p.R552K mutation in exon 11 of the SOS1 gene.Dental problems like supernumary teeth might be seen in NS patients.It is necessary to collaborate with pediatric dentist during the follow-up and treatments of these cases.