Abnormal development of circular muscles in aganglionic segments of HSCR patients

Research Square (Research Square)(2023)

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摘要
Abstract Background Hirschsprung’s disease (HSCR) is one of the most common malformations of the digestive tract. Patients with HSCR frequently manifest as having severe constipation and abdominal distension. The primary pathological feature of HSCR is the absence of ganglion cells in the distal bowel, and the arrangement of the circular and longitudinal muscles of the aganglionic segments is disorganized. To function properly, it requires an intact muscular layer as well as a neural network connection. Previous research has suggested that HSCR is a neurological disorder; however, HSCR may also be a muscular cell disorder of the intestinal smooth muscle. Methods To investigate the development rules of circular and longitudinal muscles and to research whether ENCCs affect smooth muscle function. αSMA immunohistochemistry was used to stain tissues of HSCR patients and HSCR model mice at different developmental stages. Results Under normal circumstances, the formation of circular muscles is later than that of longitudinal muscles, and the expression of αSMA in circular muscles is much lower than that in longitudinal muscles. The expression of αSMA in anganglionic segments of HSCR patients is much higher than that in the distal colon of normal control children. Conclusions Loss of ENCCs may influence the function of the circular muscles, αSMA is a biomarker for detecting the abnormal smooth muscle cell in Hirschsprung's disease patients' aganglionic segments.
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关键词
circular muscles,aganglionic segments,abnormal development
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