Medical University of Varna, Varna, Bulgaria.
Mdical University of Sofia, Sofia, Bulgaria.
Folia Med (Plovdiv). 2021 Feb 28;63(1):138-141. doi: 10.3897/folmed.63.e52763.
16p11.2 duplication syndrome is a rare disorder, often associated with intellectual disability, attention deficit, hyperactivity disorder, and a predisposition to epilepsy and schizophrenia. There are no specific dysmorphic features for this genetic condition, but micro-cephaly, micrognathia and hypertelorism could be present. We report a case of 16p11.2 duplication syndrome which has the typical clinical presentation - slight facial dysmorphism, impaired intellectual development, and autistic behavior. Whole-exome sequencing was performed, but no pathogenic or likely pathogenic mutations were identified. Array comparative genomic hybridization analysis established the diagnosis of 16p11.2 duplication syndrome, which illustrates the importance of this method when diagnosing children with unexplained intellectual disability.
16p11.2 重复综合征是一种罕见疾病,常伴有智力障碍、注意力缺陷、多动障碍以及癫痫和精神分裂症的易感性。这种遗传疾病没有特定的畸形特征,但可能存在小头畸形、小下颌和眼距过宽。我们报告了一例 16p11.2 重复综合征病例,其具有典型的临床表现——轻微的面部畸形、智力发育受损和自闭症行为。进行了全外显子组测序,但未发现致病或可能致病的突变。阵列比较基因组杂交分析确立了 16p11.2 重复综合征的诊断,这说明了在诊断不明原因智力障碍的儿童时,这种方法的重要性。