Pavone Piero, Praticò Andrea D, Campisi Corrado, Falsaperla Raffaele
Unit of Pediatrics, University Hospital OVE-Policlinico, Catania, Italy.
Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy.
BMJ Case Rep. 2016 Apr 18;2016:10.1136/bcr-2016-214388. doi: 10.1136/bcr-2016-214388.
Chromosome 10q23 contains several genes, includingPTENandBMPR1A, the mutations or microdeletion of which are associated with aggressive polyposis and malignancies in children. Deletions in this chromosomal region have also been associated with heart anomalies, developmental delay and macrocephaly. Most of the cases reported involve thePTENandBMPR1Agenes, usually associated with complex and severe anomalies. We report a case of a boy with a de novo interstitial microdeletion in 10q23.1-q23.2 spanning 6.7 Mb with boundaries from 82 087 077 to 88 847 906, not includingPTENandBMPR1A Clinical features consisted of mildly dysmorphic facies, frontal telangiectasias, poor scholastic performance and hyperactivity. Furthermore, the boy presented toe anomalies, which appeared to be novel features associated with 10q23 deletion. Further observations of 10q23.1-q23.2 deletions are necessary to confirm the clinical features observed in the proband, and to show that deletion or mutations not involvingPTENandBMPR1Amay not be associated with severe neurological impairment and malformation anomalies.
10号染色体q23区域包含多个基因,包括PTEN和BMPR1A,其突变或微缺失与儿童侵袭性息肉病和恶性肿瘤相关。该染色体区域的缺失还与心脏异常、发育迟缓及巨头畸形有关。报道的大多数病例涉及PTEN和BMPR1A基因,通常与复杂且严重的异常相关。我们报告1例男孩,其10q23.1 - q23.2区域存在一个6.7兆碱基的新发间质性微缺失,边界为82087077至88847906,不包括PTEN和BMPR1A。临床特征包括轻度面容畸形、额部毛细血管扩张、学业成绩差及多动。此外,该男孩存在足趾异常,这似乎是与10q23缺失相关的新特征。有必要对10q23.1 - q23.2缺失进行进一步观察,以确认先证者中观察到的临床特征,并表明不涉及PTEN和BMPR1A的缺失或突变可能与严重神经功能损害及畸形异常无关。