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Sonic hedgehog基因及Gli家族在人类食管闭锁并气管食管瘘中的表达

Sonic hedgehog and Glis famliy expression in the human neonatal of congenital esophageal atresia and tra- cheoesophageal fistula
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摘要 目的研究Sonichedgehog基因及Gli家族在人类先天性食管闭锁并气管食管瘘(e—sophagealatresiaandtracheoesophagealfistula,EATEF)的表达特点,探讨EA-TEF病因及发病机制的可能影响因素。方法食管吻合术中留取22例EA-TEF患儿近端食管盲端及远端气管食管瘘管组织,7例行HE染色,10例行realtimeRT-PCR处理,5例行免疫荧光染色处理。观察食管盲端及气管食管瘘管形态上的变化及各指标的差异。结果①形态学:瘘管组织内皮下可见粘液腺体,肌层稀疏且肌肉组织结构紊乱;②Shh表达:食管盲端组织中可见表达,瘘管组织中未有表达;③Glis表达:Gli-1、Gli-3mRNA表达无差异,Gli-2mRNA表达差异有统计学意义(P〈0.05),瘘管中表达低于食管盲袋。结论气管食管瘘组织具有气管源性特征。EA-TEF的发生可能与Shh信号通路表达下调有关。Gli-2的功能缺失在EA-TEF的发生中可能发挥重要作用。 Objective To describe the differential expression of Shh and Gli family members between normal proximal esophageal pouch and distal tracheoesopbageal fistula (EA-TEF). Methods Twenty twohuman proximal esophageal pouch and distal fistula samples were obtained at the time of standard repair of EA-TEF. Seven samples were processed for HE, ten for real-time reverse-transcriptase polymerase chain reaction (RT-PCR), and five for immunohistoehemistry, llesults Shh was confirmed to be present by immu- nohistochemistry in the proximal esophageal pouch, but was specifically absent in the distal fistula tract. Gli- 1 and-3 mRNA expression showed no difference in the proximal pouch and distal esophagus using real-time RT-PCR. G1F2 mRNA levels were much lower in the fistula tract than in the adjacent esophagus. Conclu- sions Abnomal expression of Shh may contribute to the malformation of EA-TEF. G1L2down-regulation may also play an important role in the mechanisms of EA-TEF malformation. These results support the conclu- sion that the fistula tract is not an esophagus-like structure .
出处 《中华小儿外科杂志》 CSCD 北大核心 2012年第5期329-332,共4页 Chinese Journal of Pediatric Surgery
基金 北京市自然科学基金资助(编号7062024)
关键词 食管闭锁 气管食管瘘 Sonic HEDGEHOG 实时荧光定量RT PCR Esophageal atresia Tracheoesophageal fistula Sonic hedgehog Real time RT- PCR
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参考文献13

  • 1李樱子,陈永卫,曾晓蓓,孙海梅,徐元芹.先天性食管闭锁并气管食管瘘的早期胚胎学研究[J].中华小儿外科杂志,2007,28(2):78-81. 被引量:7
  • 2王伟,陈永卫,孙海梅.先天性食管闭锁并气管食管瘘的大鼠胚胎学研究[J].中华小儿外科杂志,2009,30(4):242-245. 被引量:2
  • 3Crisera CA, Conndly PR, Marmureanu AR, et al. Esophageal atresia with tracheoesophageal fistula: suggested mechanism in faulty organogenesis. J Pediatr Surg , 1999,34(1): 204-2(18.
  • 4Spilde TL , Bhatia AM , Marosky JK, et al. Complete discontinuity of the distal fistula tract from the developing gut: direct histologic evidence for the mechanism of tracheoesophageal. Anat Rec , 2002,267(3) :220-224.
  • 5Crisera CA, Maldonado TS, Kadison AS, et al. Pattering of the'distal esophagus in esophageal atresia with traeheo-esophageal fistula: Is thyroid transcription factor 1 a player ? J Surg Res, 2000, 929(2):245-249.
  • 6Mendelson CR. Role of transcription factors in fetal lung development and surfactant protein gene expression. Annu Rev Physiol, 2000,62 ( 1 ): 875-915.
  • 7Spilde TL, Bhatia AM, Miller KA, et al. Thyroid transcription factor-1 expression in the human neonatal traeheoesophageal fistula. J Pediatr Surg, 2002,37(7) :1065-1067.
  • 8Dutta HK, Mathur M, Bhatnagar V. A histopathological study of esophageal atresia and tracheoesophageal fistula. J Pediatr Surg, 2000,35 (3) : 438-441.
  • 9Marigo V, Rober DJ, Lee SM. Cloning, expression, and chro mosomal loeation of SHH and IHH:two human homologues of the DrosoPhila segment Polarity gene hedgehog. Genomics, 1995,28(1) :44-51.
  • 10Kim JH, Kim PCW, Hui C-c. The VACTERL association: lessons from the sonic hedgehog pathway. Clin Genet,2001,59 (5) :306-315.

二级参考文献21

  • 1李樱子,陈永卫,曾晓蓓,孙海梅,徐元芹.先天性食管闭锁并气管食管瘘的早期胚胎学研究[J].中华小儿外科杂志,2007,28(2):78-81. 被引量:7
  • 2Braden Kuo MD, Daniela Urma MD. Esophagus anatomy and development. GI Motilily online,20116, doi: 10. 1038/gimo6.
  • 3Possogel AK, Diez-Pardo JA, Morales C, et al. Embryology of esophageal atresia in the adriamyein rat model. J Pediatr Surg, 1998, 33:606-612.
  • 4Crisera CA, Connelly PR, Marmureanu AR, et al. Esophageal atresia with tracheoesophageal fistula: suggested mechanism in faulty organogenesis. J Pediatr Surg, 1999, 34: 204-208.
  • 5Spilde TL, Bhatia AM, Marosky JK, et al. Complete disconti nuity of the distal fistula tract from the developing gut:direct his tologic evidence for the mechanism of tracheoesophageal. Anat Rec,2002, 267:220- 224.
  • 6Merei JM, Hutson JM. Embryogenesis of tracheoesophageal anomalies: a review. Pediatr Surg Int, 2002,18:319- 326.
  • 7Qi BQ, Beasley SW. Relationship of the notochord to foregut development in the fetal rat model of esophageal atresia. J Pediatr Surg, 1999,34: 1593-1598.
  • 8Orford J, Manglick P, Cass DT, et al. Mechanisms for the development of esophageal atresia. J Pediatr Surg, 2001,36: 985- 994.
  • 9Possoegel AK, Diez Pardo JA, Morales C, et al. Notochord involvement in experimental esophageal atresia. J Pediatr Surg, 1999,15 : 201 -205.
  • 10Orford J, Manglick P, Cass DT, et al. Mechanisms for the development of esophageal atresia. J Pediatr Surg, 2001,36: 985-994.

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