Department of Pediatrics, National Hospital Organization Nagasaki Medical Center, Omura, Japan; Department of Human Genetics, Atomic Bomb Disease Institute, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan; Department of Pediatrics, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
The Misakaenosono Mutsumi Developmental, Medical and Welfare Center, Nagasaki, Japan.
Gene. 2022 Aug 15;835:146663. doi: 10.1016/j.gene.2022.146663. Epub 2022 Jun 8.
Down syndrome (DS) is a common congenital disorder caused by trisomy 21. Due to the increase in maternal age with population aging and advances in medical treatment for fatal complications in their early childhood, the prevalence and life expectancy of DS individuals have greatly increased. Despite this rise in the number of DS adults, their hematological status remains poorly examined. Here, we report that three hematological abnormalities, leukopenia, macrocytosis, and thrombocytopenia, develop as adult DS-associated features. Multi- and uni-variate analyses on hematological data collected from 51 DS and 60 control adults demonstrated that young adults with DS are at significantly higher risk of (i) myeloid-dominant leukopenia, (ii) macrocytosis characterized by high mean cell volume (MCV) of erythrocytes, and (iii) lower platelet counts than the control. Notably, these features were more pronounced with age. Further analyses on DS adults would provide a deeper understanding and novel research perspectives for multiple aging-related disorders in the general population.
唐氏综合征(DS)是一种常见的先天性疾病,由 21 三体引起。由于人口老龄化导致母亲年龄增加,以及对其幼年致命并发症的医疗治疗进展,DS 个体的患病率和预期寿命大大增加。尽管 DS 成年人的数量有所增加,但他们的血液学状况仍未得到充分检查。在这里,我们报告了三种血液学异常,即白细胞减少、巨细胞增多和血小板减少,这些异常是成年 DS 相关特征。对来自 51 名 DS 患者和 60 名对照成年人的血液学数据进行的多变量和单变量分析表明,年轻的 DS 患者患(i)以骨髓细胞为主的白细胞减少症、(ii)以红细胞平均细胞体积(MCV)高为特征的巨细胞增多症、和(iii)血小板计数较低的风险明显高于对照组。值得注意的是,这些特征随着年龄的增长而更加明显。对 DS 成年人的进一步分析将为一般人群中多种与衰老相关的疾病提供更深入的了解和新的研究视角。