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为了解我国先天性膈疝的基本状况和影响患儿预后的危险因素。1986年9月~1993年12月,在全国出生缺陷监测网中监测妊娠28周至出生后7天的住院分娩围产儿。结果显示:在4777220例围产儿中,共检出321例先天性膈疝,膈疝发病率0.7/万;患儿围产期总病死率为84.1%,其中死胎、死产占28.1%,新生儿7天内死亡占71.9%。围产期死亡与存活病例在出生体重上存在显著性差异。54.5%的患儿伴有其他畸形;死亡患儿中伴发心、肺和中枢神经系统严重畸形所占的比例最大,是引起患儿死亡的重要因素。单发畸形病例病死率为74.7%,多发畸形病例为92.0%,两者有显著性差异。经产前诊断的患儿占11.3%,以多发畸形所占比例大。说明我国膈疝畸形产前诊断率低,畸形严重,患儿围产期病死率高,预后差。
To understand the basic condition of congenital diaphragmatic hernia in our country and the risk factors affecting the prognosis of children. From September 1986 to December 1993, the perinatal delivery was monitored in the National Birth Defects Monitoring Network from 28 weeks of gestation to 7 days of birth. The results showed that in 4777220 cases of perinatal children, a total of 321 cases of congenital diaphragmatic hernia were found. The incidence of diaphragmatic hernia was 0.7 / 10,000. The total perinatal mortality was 84.1%. The stillbirth and stillbirth accounted for 28%. 1%, neonatal death within 7 days accounted for 71.9%. There was a significant difference in birth weight between perinatal death and survivors. 54.5% of children with other deformities; children with death associated with heart, lung and central nervous system, the largest proportion of serious malformations, is an important factor in causing death in children. Case fatality rate of single malformations was 74.7%, multiple malformations cases were 92.0%, both significant differences. Prenatal diagnosis of children accounted for 11.3%, with a large proportion of multiple malformations. This shows that our country diaphragmatic hernia deformity prenatal diagnosis rate is low, deformity is severe, perinatal mortality in children with high, poor prognosis.