Imataka George, Suzumura Hiroshi, Arisaka Osamu
Department of Pediatrics, Dokkyo Medical University School of Medicine, Shimotsuga, Tocihgi 321‑0293, Japan.
Mol Med Rep. 2016 Mar;13(3):2457-66. doi: 10.3892/mmr.2016.4806. Epub 2016 Jan 22.
Trisomy 18 syndrome is a common autosomal aneuploidy chromosomal abnormality caused by the presence of extra chromosome 18 that leads to malformations of various parts of the body. In this study, we retrospectively investigated the effect of the medical progression and prognosis of 44 cases of trisomy 18, admitted to our neonatal intensive care unit between 1992 and 2013. The patients were divided into group A (n=20, 1992‑2002) and group B (n=24, 2003‑2012). Following delivery, karyotype, gender, gestational weeks, birth place, cesarean section, Apgar score and birth weight were analyzed using the Fisher's exact test, unpaired t‑test and Mann‑Whitney U test. Based on the statistical results, a comparison was made of the two groups and no significant differences were observed. Clinical data of major complications, mechanical ventilation, discharge from hospital and survival days were reviewed for the cases of trisomy 18. Of the 44 patients, 42 had cardiac anomaly, 16 had esophageal atresia, and 3 patients had brain anomaly. Ventilation treatment was performed in 29 cases (65.9%) and an increased percentage was identified in group B patients. The percentage survival was estimated using Kaplan‑Meier curves and the two groups were analyzed using the generalized Wilcoxon test. Improvement in life prognosis was observed in group B as compared to group A. The log‑rank test was used to assess survey periods of 180 days, 1 year, and the entire observation period. Although significant differences were observed for the prognosis of trisomy 18 at 180 days after birth, after 1 year and the entire survey period after birth, the significant differences were not confirmed. In conclusion, results of the present study provide information concerning genetic counseling for parents/guardians and life prognosis, prior to applying intensive management to newborns with trisomy 18.
18三体综合征是一种常见的常染色体非整倍体染色体异常疾病,由额外的18号染色体导致身体各部位畸形。在本研究中,我们回顾性调查了1992年至2013年间入住我院新生儿重症监护病房的44例18三体综合征患者的医疗进展及预后情况。患者分为A组(n = 20,1992 - 2002年)和B组(n = 24,2003 - 2012年)。分娩后,采用Fisher精确检验、非配对t检验和Mann - Whitney U检验对核型、性别、孕周、出生地、剖宫产、阿氏评分和出生体重进行分析。根据统计结果,对两组进行比较,未观察到显著差异。回顾了18三体综合征患者的主要并发症、机械通气、出院及存活天数等临床资料。44例患者中,42例有心脏畸形,16例有食管闭锁,3例有脑部畸形。29例(65.9%)患者接受了通气治疗,B组患者的比例有所增加。采用Kaplan - Meier曲线估计生存率,并使用广义Wilcoxon检验对两组进行分析。与A组相比,B组患者的生命预后有所改善。采用对数秩检验评估出生后180天、1年及整个观察期。虽然在出生后180天、1年后及整个观察期18三体综合征的预后存在显著差异,但差异未得到证实。总之,本研究结果为18三体综合征新生儿进行强化管理前为父母/监护人提供遗传咨询及生命预后信息。