无脾综合征伴复杂性先天性心脏病1例报道并文献复习  

Asplenia syndrome with complex congenital heart disease:a case report and literature review

作　　者：李娜[1] 黄玉柱[1] 高奎武[1] 孙景巍[1] 

机构地区：[1]安徽省蚌埠市第一人民医院儿内科,233000

出　　处：《淮海医药》2016年第6期661-662,共2页Journal of Huaihai Medicine

摘　　要：目的:提高临床医生对于无脾综合征的认识,帮助及早诊断与治疗。方法:报道1例3岁1月无脾综合征伴复杂性先天性心脏病患儿,结合文献资料复习无脾综合征的病因、发病机制、临床表现、诊断及预防治疗。结果:本病病因不明;临床表现无脾综合征发病率低,预后差。出生时即有青紫、气急;先天性脾发育不全或缺如,胸腹腔器官结构和位置异常;心血管系统严重发育畸形。外周血涂片找到Howell-Jolly小体常具有诊断意义。本病目前缺乏有效的治疗手段。结论:孕期预防某些致畸因子的影响、及时进行产前检查,进行必要的干预以预防无脾综合征的发生、及时手术矫治提高生命质量。Objective： To improve the recognition of asplenia syndrome,which is beneficial to early diagnosis and treatment. Methods： A case of a 3-year-old child patient of asplenia syndrome with complex congenital heart disease was studied and related literature was reviewed in terms of the etiology,pathogenesis,clinical manifestations,diagnosis,prevention and treatment. Results： Asplenia syndrome is a rare congenital disease with poor prognosis. Patients with the disease tend to be born with bruising,short of breath,congenital spleen hypoplasia or absence,abnormal structure and position of the thoracic and abdominal organs. Severe cardiovascular system malformation is often present. Finding Howell-Jolly small body in peripheral blood is considered to be diagnostic. There is currently a lack of effective treatments. Conclusion： Preventing some teratogenic factors and taking prenatal test timely may contribute to the prevention of asplenia syndrome. Operative correction can also be used to improve patients＇ quality of life.

关 键 词：无脾综合征 先天性心脏病 

分 类 号：R657.6[医药卫生—外科学] R541[医药卫生—临床医学]