Sonic hedgehog and Glis famliy expression in the human neonatal of congenital esophageal atresia and tracheoesophageal fistula

Abstract

Objective To describe the differential expression of Shh and Gli family members between normal proximal esophageal pouch and distal tracheoesophageal 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 immunohistochemistry.Results Shh was confirmed to be present by immunohistochemistry in the proximal esophageal pouch,but was specifically absent in the distal fistula tract.Gli1and-3 mRNA expression showed no difference in the proximal pouch and distal esophagus using real-time RT-PCR.Gli-2 mRNA levels were much lower in the fistula tract than in the adjacent esophagus.Conclusions Abnomal expression of Shh may contribute to the malformation of EA-TEF.Gli2down-regulation may also play an important role in the mechanisms of EA-TEF malformation.These results support the conclusion that the fistula tract is not an esophagus-like structure. Key words: Esophageal atresia; Tracheoesophageal fistula; Sonic hedgehog; Real-time RTPCR