Division of Pathology and Laboratory Medicine, Phoenix Children's Hospital, Phoenix, Arizona, USA.
Department of Child Health, University of Arizona College of Medicine-Phoenix, Phoenix, Arizona, USA.
Am J Med Genet A. 2023 Sep;191(9):2392-2397. doi: 10.1002/ajmg.a.63350. Epub 2023 Jul 12.
15q26 deletion is a rare genomic disorder characterized by intrauterine and postnatal growth retardation, microcephaly, intellectual disability, and congenital malformations. Here, we report a 4-month-old female with intrauterine growth retardation, short stature, pulmonary hypertension, atrial septal defect and congenital bowing of long bones of the legs. Chromosomal microarray analysis showed a de novo deletion of approximately 2.1 Mb at 15q26.3 region that does not include IGF1R. Our analysis of patients documented in the literature and the DECIPHER database with 15q26 deletions distal to IGF1R, including 10 patients with de novo pure deletions, allowed us to define the smallest region of overlap to 686 kb. This region includes ALDH1A3, LRRK1, CHSY1, SELENOS, SNRPA1, and PCSK6. We propose haploinsufficiency of one or more genes, besides IGF1R, within this region may contribute to the clinical findings in patients with 15q26.3 deletion.
15q26 缺失是一种罕见的基因组疾病,其特征为宫内和产后生长迟缓、小头畸形、智力障碍和先天性畸形。在此,我们报告了一例 4 月龄女性,存在宫内生长迟缓、身材矮小、肺动脉高压、房间隔缺损和下肢长骨先天性弓形。染色体微阵列分析显示 15q26.3 区域存在约 2.1Mb 的新生缺失,不包括 IGF1R。我们对文献和 DECIPHER 数据库中记录的 IGF1R 远端 15q26 缺失患者进行分析,包括 10 例新发纯缺失患者,从而确定最小的重叠区域为 686kb。该区域包括 ALDH1A3、LRRK1、CHSY1、SELENOS、SNRPA1 和 PCSK6。我们提出,该区域内除 IGF1R 外,一个或多个基因的杂合性缺失可能导致 15q26.3 缺失患者的临床表现。