Miao Zong-Yu, Chen Shi-Feng, Wu Hong, Liu Xiao-Yan, Shao Hui-Yuan
Medical Laboratory, Yantai Yu Huang Ding Hospital, 20#, The East Road of Yu Huang Ding, Zhifu District, Yantai, 264000, Shandong, China.
Open Life Sci. 2022 Apr 26;17(1):416-425. doi: 10.1515/biol-2022-0046. eCollection 2022.
Chromosomal abnormality is one of the important causes of dysplasia in children. However, due to regional and ethnic differences, the reported rates of chromosomal abnormalities in patients with dysplasia vary greatly. Moreover, the clinical manifestations in children with rare chromosomal diseases were heterogeneous. So, we retrospectively analyzed the karyotype results of 436 children with dysplasia and conducted a detailed analysis of rare chromosomal diseases. The results showed that chromosomal abnormalities were present in 181 of 436 cases. Intellectual disability, dysmorphology, congenital malformations, the disorder of sexual development, and short stature were the main five clinical symptoms in children with chromosomal abnormalities. Moreover, 136 cases of Trisomy 21 (Tri21) were detected, of which 130 were standard Tri21, 5 were robertsonian Tri21, and 1 was chimera type. In addition, 16 cases of rare abnormal karyotype, including complex Tri21, complex Turner syndrome, 4p-syndrome, 18q-syndrome, and 5p-syndrome, were also detected. In summary, chromosome abnormality is one of the important causes of dysplasia in children. Furthermore, prenatal screening and diagnosis could play a great significance in preventing dysplasia in children. In addition, the retrospective analysis of rare cases is valuable for clinical diagnosis and risk assessment of recurrence.
染色体异常是儿童发育异常的重要原因之一。然而,由于地区和种族差异,发育异常患者中染色体异常的报告发生率差异很大。此外,罕见染色体疾病患儿的临床表现具有异质性。因此,我们回顾性分析了436例发育异常儿童的核型结果,并对罕见染色体疾病进行了详细分析。结果显示,436例中有181例存在染色体异常。智力残疾、形态异常、先天性畸形、性发育障碍和身材矮小是染色体异常患儿的主要五种临床症状。此外,检测到136例21三体(Tri21),其中130例为标准Tri21,5例为罗伯逊易位Tri21,1例为嵌合型。此外,还检测到16例罕见的异常核型,包括复杂Tri21、复杂特纳综合征、4p综合征、18q综合征和5p综合征。综上所述,染色体异常是儿童发育异常的重要原因之一。此外,产前筛查和诊断对预防儿童发育异常具有重要意义。此外,对罕见病例的回顾性分析对临床诊断和复发风险评估具有重要价值。