Ohta Sayaka, Isojima Tsuyoshi, Mizuno Yoko, Kato Motohiro, Mimaki Masakazu, Seki Masafumi, Sato Yusuke, Ogawa Seishi, Takita Junko, Kitanaka Sachiko, Oka Akira
Department of Pediatrics, Graduate School of Medicine, University of Tokyo, Tokyo, Japan.
Department of Pediatrics, Teikyo University School of Medicine, Tokyo, Japan.
Pediatr Int. 2017 Jan;59(1):99-102. doi: 10.1111/ped.13181.
Partial monosomy of 10p is a rare chromosomal abnormality. Common features are hypoparathyroidism, deafness, renal anomalies, distinctive facies, and mental retardation, with phenotypic variability. We report two patients with chromosomal abnormalities identified on single-nucleotide polymorphism (SNP) array analysis. Although patient 1 had common features of monosomy10p, G-banding indicated a normal karyotype. SNP array and fluorescence in situ hybridization (FISH), however, indicated unbalanced translocation of a 10p terminal deletion of 11.7 Mb and a 15q terminal duplication of 8.2 Mb. In patient 2, SNP array and FISH indicated a 10p terminal deletion of 12.6 Mb and a 7q terminal duplication of 1.9 Mb. This is the first case report of monosomy 10p combined with trisomy 15q (patient 1). Because the clinical heterogeneity of the 10p deletion syndrome would be affected by duplication of another chromosome, we emphasize that SNP/microarray analysis is necessary to confirm genotype-phenotype correlation.
10p部分单体性是一种罕见的染色体异常。常见特征包括甲状旁腺功能减退、耳聋、肾脏异常、特殊面容和智力发育迟缓,且表型存在变异性。我们报告了两名通过单核苷酸多态性(SNP)阵列分析鉴定出染色体异常的患者。虽然患者1具有10p单体性的常见特征,但G显带显示核型正常。然而,SNP阵列和荧光原位杂交(FISH)表明存在不平衡易位,即10p末端缺失11.7 Mb以及15q末端重复8.2 Mb。在患者2中,SNP阵列和FISH表明10p末端缺失12.6 Mb以及7q末端重复1.9 Mb。这是10p单体性合并15q三体性的首例病例报告(患者1)。由于10p缺失综合征的临床异质性会受到另一条染色体重复的影响,我们强调SNP/微阵列分析对于确认基因型与表型的相关性是必要的。