It may due to the complex genes regulatory to ENCCs in children with HSCR.1 5 9 Although many genes have been identified to be associated with HSCR,2 11 such as RET, EDNRB, RARB, GATA2, and SOX10, which commonly regulate ENCCs during the development of ENS, how the TRN contributes to HSCR pathogenesis remains to be investigated. This evidence concerns the gene TNPO1 and Hirschsprung disease.