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目的对唐氏综合征(DS)中先天性心脏病(CHD)相关情况进行监测分析,探讨DS和CHD的关系。方法回顾性分析2002年1月-2014年12月在该院诊断的203例DS患儿的临床资料,根据患儿是否合并CHD分为DS不合并CHD组(122例)和DS合并CHD组(81例),比较两组孕妇和患儿的基本情况,分析DS合并CHD的患病率、类型与CHD类型之间的关系等。结果两组孕妇年龄、孕次构成情况差异无统计学意义(P>0.05),学历构成比情况差异有统计学意义(P<0.05)。两组DS患儿的性别构成情况差异无统计学意义(P>0.05),两组胎龄、转归、确诊时间、检查方法差异均有统计学意义(P<0.001)。81例(39.90%)DS合并CHD患儿中间隔类缺损43例(53.09%),包括室间隔缺损20例(24.69%),房间隔缺损14例(17.28%),房室间隔缺损4例(4.94%),复合间隔类缺损5例(6.17%);非间隔缺损类CHD(包括动脉导管未闭、法洛氏四联症、右心室双出口、肺动脉闭锁等)38例(46.91%)。年龄≥35岁患者的CHD类型以间隔缺损类为主,年龄<35岁组患者的CHD类型以非间隔缺损类为主,不同年龄CHD类型构成比差异有统计学意义(χ~2=12.908,P=0.045)。DS不同核型与CHD类型差异无统计学意义(χ~2=3.511,P=0.991)。18例合并其他畸形,如消化道畸形、骨骼系统畸形等。结论 DS与CHD的发生密切相关。DS合并CHD的类型以间隔类缺损为主,最常见的CHD类型为动脉导管未闭,其次为室间隔缺损。DS染色体核型与CHD类型无关。
Objective To monitor the incidence of congenital heart disease (CHD) in Down Syndrome (DS), and to explore the relationship between DS and CHD. Methods The clinical data of 203 children with DS diagnosed in our hospital from January 2002 to December 2014 were retrospectively analyzed. According to whether children with CHD or not had CHD, there were 122 cases with DS without CHD and 40 cases with DS with CHD 81 cases), the basic situation of two groups of pregnant women and children were compared, the prevalence of CH combined with DS, the type and type of CHD relationship between. Results There were no significant differences in the age and gestational age between the two groups (P> 0.05). The educational composition was significantly different (P <0.05). There was no significant difference in sex composition between the two groups of children with DS (P> 0.05). There was significant difference in gestational age, outcome, diagnosis time and examination between the two groups (P <0.001). There were 43 cases (53.09%) of septal defects in 81 cases (39.90%) DS patients with CHD, including 20 cases of ventricular septal defect (24.69%), 14 cases (17.28%) of atrial septal defect and 4 cases of atrioventricular septal defect 4.94%). There were 5 cases (6.17%) of composite interval defect. 38 cases (46.91%) of non-septal defect CHD including patent ductus arteriosus, tetralogy of Fallot, double outlet of right ventricle and pulmonary atresia. The type of CHD in patients aged 35 years and older was mainly of the type of septal defect. The CHD types of patients aged <35 years old were mainly non-septal defects, and the proportions of CHD types at different ages were statistically significant (χ ~ 2 = 12.908, P = 0.045). There was no significant difference between different karyotypes and CHD types in DS (χ ~ 2 = 3.511, P = 0.991). 18 cases combined with other malformations, such as gastrointestinal malformations, skeletal system deformities and so on. Conclusions DS is closely related to the occurrence of CHD. The types of CHD combined with CHD were mostly discontinuous. The most common type of CHD was patent ductus arteriosus, followed by ventricular septal defect. DS chromosome karyotype and CHD type has nothing to do.