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

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 .