la Cour Sibbesen Else, Jespersgaard Cathrine, Alosi Daniela, Bisgaard Anne-Marie, Tümer Zeynep
Applied Human Molecular Genetics, Kennedy Center, Copenhagen University Hospital, Rigshospitalet, Glostrup, Denmark.
Am J Med Genet A. 2013 Jun;161A(6):1447-52. doi: 10.1002/ajmg.a.35901. Epub 2013 Apr 30.
In this report, we describe a female child with dysmorphic features and developmental delay. Chromosome microarray analysis followed by conventional karyotyping revealed a ring chromosome 9 with a 12 Mb deletion at 9pter-p23 and a 540 kb deletion at 9q34.3-qter. Four percent of the analyzed cells had monosomy 9. The patient has the features of both the Kleefstra syndrome and the chromosome 9p-syndrome, including trigonocephaly, long philtrum, hypertelorism, and retro-/micronagthia. The deletion of the patient overlaps with several of the proposed critical regions for the 9p deletion syndrome.
在本报告中,我们描述了一名具有畸形特征和发育迟缓的女童。染色体微阵列分析及随后的常规核型分析显示,9号环状染色体在9pter - p23处有12 Mb的缺失,在9q34.3 - qter处有540 kb的缺失。4%的分析细胞存在9号染色体单体性。该患者具有克莱夫斯特拉综合征和9p染色体综合征的特征,包括三角头畸形、长人中、眼距过宽以及后缩/小颌畸形。患者的缺失区域与9p缺失综合征的几个拟关键区域重叠。