Petrova O V, Murygina O I, Shashin S A, Nikulina D M, Tarasov D G
Federal Center for Cardiovascular Surgery, Astrakhan, Russia.
Astrakhan State Medical University, Astrakhan, Russia.
Khirurgiia (Mosk). 2020(12):111-114. doi: 10.17116/hirurgia2020121111.
To report a rare case of Down's syndrome with congenital acute leukemia and congenital heart disease.
We have retrospectively analyzed an electronic database of patients aged 0-17 years who underwent inpatient treatment of congenital heart disease at the Federal Center for Cardiovascular Surgery for the period from January 1, 2010 to December 31, 2018. Incidence of Down's syndrome in children with congenital heart defects was 5.36% of the total number of children with congenital heart defects. No gender differences were identified. The most common congenital heart defects were ventricular septal defect, atrioventricular septal defect, atrial septal defect, patent ductus arteriosus, tetralogy of Fallot. Down's syndrome and congenital heart disease were diagnosed prenatally in 33 out of 319 patients. A rare case of Down's syndrome combined with congenital heart disease and acute leukemia is described. Treatment outcome was unfavorable.
CONCLUSION. S: Evere concomitant diseases in a child with Down's syndrome determine high postoperative morbidity and mortality. Prenatal diagnosis of Down's syndrome via screening of pregnant women for ultrasound and biochemical markers of this pathology is required.
报告1例患有先天性急性白血病和先天性心脏病的唐氏综合征罕见病例。
我们回顾性分析了2010年1月1日至2018年12月31日期间在联邦心血管外科中心接受先天性心脏病住院治疗的0至17岁患者的电子数据库。先天性心脏缺陷患儿中唐氏综合征的发病率占先天性心脏缺陷患儿总数的5.36%。未发现性别差异。最常见的先天性心脏缺陷是室间隔缺损、房室间隔缺损、房间隔缺损、动脉导管未闭、法洛四联症。319例患者中有33例在产前被诊断出患有唐氏综合征和先天性心脏病。本文描述了1例唐氏综合征合并先天性心脏病和急性白血病的罕见病例。治疗结果不佳。
唐氏综合征患儿严重的合并症决定了术后高发病率和高死亡率。需要通过对孕妇进行超声检查和该病症的生化标志物筛查来对唐氏综合征进行产前诊断。
