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新生儿VACTERL联合征 被引量:3

VACTERL Association in Neonates
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摘要 目的 :提高对新生儿VACTERL联合征的诊断和治疗水平。方法 :回顾分析食道闭锁 12例和肛门闭锁 2 8例 ,按VACTERL联合征的诊断标准 ,六种畸形中至少有三种畸形同时存在时 ,就可诊为VACTERL联合征。结果 :共有 11例诊为VACTERL联合征 ,诊断时间最早为 2h ,最迟为 2 2d ,平均诊断年龄 3d。肛门闭锁半瘘 9例 ,并食道闭锁 7例 ,并先心 7例 ,并椎体畸形 6例 ,并泌尿生殖系畸形 5例 ,合并肢体畸形 3例。 11例中 6例手术治疗 ,5例成活 ,1例死亡 ,另 5例放弃治疗。 5例随访 6个月至 2年 ,均健康。结论 :VACTERL联合征是一组少见的先天性畸形 ,早期诊断很有必要 ,尤其是食道闭锁的诊断临床上应有足够的认识 ;积极治疗可获得正常的生存。 Objective: To elevate the levels of diagnosis and treatment in neonate VACTERL association.Methods:12 cases of esophageal atresia and 28 cases of anal atresia in our hospital from 1999 to 2001 were retrospectively analyzed, according to the diagnostic criterion of VACTERL association.If there were at least 3 or more of 6 malformations,one could be diagnosed as the VACTERL association.Results:There were 11 cases of diagnosed VACTERL association.Time of early diagnosis was in 2 hours and lasted into 22 days,with an average time of 3 days.There were 9 cases of anal atresia or fistula,7 cases of esophageal atresia,7 cases of congenital heart diseases,and 6 cases of limb malformations.Of 11 cases, 6 cases were operated,5 survivals and 1 death.The other 5 cases were given up.All 5 survival cases had good health as follow up for 6 month to 2 years.Conclusion:VACETERL association is a group of rate congenital malformations.Early diagnosis is important,especially to detect some problems related to esophageal atresia.Following treatment actively can obtain normal survivals.
出处 《江西医学院学报》 CAS 2002年第4期82-84,共3页 Acta Academiae Medicinae Jiangxi
关键词 新生儿 VACTERL联合征 先天性畸形 诊断 治疗 neonate congenital malformation VACTERAL association
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参考文献4

  • 1MeMullen KP,Karnes Ps,Moir CR,et al.Familial recurrence of tracheoesophageal fistula and associated associated malformations[J].Am J Med Genet 1996,63(4):525-528.
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二级参考文献1

  • 1周自永 王世铮.新编常用药物手册[M].北京:金盾出版社,1998.48.

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同被引文献7

  • 1QuanL, Smith DW. The VATERL association ; vertebral defects, anal atrsia, trecheoesophageal fistula with esophageal atrsia, radial dysplasi- a,Birth Defects Orig[ J]. Art Ser,1972,8(2) : 75 -78.
  • 2Rittler M, Paz JE, Caastilla EE. VACTERL association, epidemiologic definition ang delineation [ J ]. Am J Med Genet, 1006,63 (4) :529.
  • 3Townes PL, Brocks ER. Hereditary syndrome of imperforate anus with hand, foot and ear anomalies [ J ]. J Pediatr, 1972,81 ( 2 ) :321 - 326.
  • 4Rittler M,Paz J E,Castilla E E.VACTERL association, epidemiologic definition and delineation. American journal of medical genetics . 1996
  • 5郭庆强,吴秀蓉,钟美珍,吴虎圣.新生儿VACTERL联合征的临床与影像学表现[J].中国新生儿科杂志,2014,29(3):149-152. 被引量:5
  • 6秦凤真,李胜利,文华轩,姚远,毕静茹,袁鹰,余蓉.VACTERL联合征的产前超声诊断分析[J].中华医学超声杂志(电子版),2014,11(7):20-25. 被引量:14
  • 7吴秋萍,吴怡翔,梁忠杰,李昌崇,陈尚勤.新生儿VACTERL联合征33例临床特点及预后分析[J].中华新生儿科杂志(中英文),2017,32(4):278-282. 被引量:4

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