Hirschsprung disease (HSCR) is the most prevalent congenital gut motility condition, defined by functional intestinal obstruction due to a severe abnormality of the enteric neural system and involves severe abnormalities of the enteric nervous system. The goal of this study was to look at the distribution patterns of collagens I (Col I), III (Col III), and IV (Col IV) in the enteric nervous system of HSCR patients to see if collagen levels in the aganglionic colon were altered. Col I, Col III, and Col IV expression levels in colonic muscle were examined in 129 HSCR children. The immunofluorescence labeling was used to examine the morphological localization of the three collagens and the myenteric ganglia. To investigate the relative amounts of these collagens in aganglionic, transitional, and ganglionic colon segments, western blots and real-time fluorescence quantitative polymerase chain reaction were used. Col I and Col III immunoreactivities were high in the ganglionic section around and within myenteric ganglia, moderate in the transitional segment, and modest in the aganglionic region. Col IV immunoreactivity followed the reverse trend, with the ganglionic segment having the lowest levels and the aganglionic section having the greatest levels.
Col I, III, and IV levels are lower in the distal colon of HSCR patients, whereas Col IV levels are higher.
