Dentofacial Features in Schimke Immuno-Osseous Dysplasia: From Childhood to Adolescence

Abstract

Schimke immune-osseous dysplasia (SIOD) is a sporadic multi-system disorder mainly characterized by spondyloepiphyseal dysplasia, immune insufficiency, and renal failure. Little evidence is available regarding the dentofacial features of SIOD. This study presents the clinical course of a 16-year-old girl suffering from SIOD, focusing on her oral anomalies from her mixed to permanent dentition. After kidney transplantation and immune therapy, the patient discussed herein is supposed to live longer than a majority of the Schimke population. These findings might play a role in the earlier diagnosis of the syndrome and can better emphasize oral care throughout their lives. This is the first study that thoroughly discusses the dental anomalies of an SIOD patient from her mixed to permanent dentition.