Bayat Allan, Møller Lisbeth Birk, Hjortshøj Tina Duelund
Ugeskr Laeger. 2018 Mar 12;180(11).
KBG syndrome is a rare condition characterised by macrodontia of the upper central incisors, distinctive craniofacial findings, short stature, skeletal anomalies, and neurologic involvement including global developmental delay, seizures, and intellectual disability. This is a case report of a seven-year-old boy, who presented with symptoms fulfilling the diagnostic criteria of KBG syndrome, molecularly confirmed by detection of a heterozygous mutation in ANKRD11. To our knowledge, this is the first patient diagnosed with KBG syndrome in Denmark. The aim of this study is to raise awareness of this recognisable syndrome.
KBG综合征是一种罕见疾病,其特征为上颌中切牙巨大、独特的颅面表现、身材矮小、骨骼异常以及神经受累,包括全面发育迟缓、癫痫发作和智力残疾。本文报告了一名7岁男孩的病例,其症状符合KBG综合征的诊断标准,并通过检测ANKRD11基因中的杂合突变在分子层面得到确诊。据我们所知,这是丹麦首例被诊断为KBG综合征的患者。本研究的目的是提高对这种可识别综合征的认识。