Abstract：Objective To investigate the immunohistochemical characteristics of SOX-10 and GDNF in different intestinal tracts of children with Hirschsprung's disease (HD).Methods The clinical data of 30 children with HD from our Hospital were selected. The specimens of dilated segment, transitional segment and narrow segment were taken from the observation group. At the same time, 10 children who underwent colostomy due to anal atresia in the same period were selected as the control group, and routine pathological examination was performed. The expression of SOX-10,GDNF protein in the intestine of the two groups was detected by immunohistochemistry. The relationship between its expression and clinicopathological features was analyzed.Results Among 30 cases of HD, SOX-10 positive cells were not found in 28 cases of narrow segment submucosal and myenteric plexus, but only a little expression was found in 2 cases. In the normal group, the weakly positive, positive or strongly positive ganglia and glial cells were observed in the intestinal canal, HD dilated segment and transitional segment, respectively, but the expression intensity of SOX-10 was also different among the groups. The expression area of SOX-10 nerve plexus in the normal group was larger than that in the transitional group, but the expression of SOX-10 nerve plexus in narrow segment was not clear. The cumulative optical density of SOX-10 positive expression in HD dilated segment, HD transitional segment and HD narrow segment was significantly lower than that in the normal control group (P<0.05). GDNF antibodies were mainly expressed in ganglion cells, nerve fibers and smooth muscle cells. GDNF was positive in ganglion cells and smooth muscle cells, but the expression intensity of ganglion cells was higher than that of smooth muscle cells. Ganglion cells or smooth muscle cells were positive in all specimens, but the expression intensity of GDNF in ganglion cells was stronger than that in smooth muscle cells. In the narrow segment, the positive expression of GDNF was seen in nerve fibers proliferation. The shape of nerve fibers was wavy and the radius of nerve fibers was larger than that of other groups.Conclusions The expression of SOX-10 and GDNF protein in diseased colon of children with HD was significantly lower than that in normal and dilated colon.
陈钦明, 吴凯, 王健俊, 何继贤, 杨六成. 先天性巨结肠组织中SOX-10、GDNF表达及意义[J]. 医学临床研究, 2019, 36(2): 233-233.
CHEN Qin-ming, WU Kai, WANG Jian-jun, et al. Expression and Significance of SOX-10 and GDNF in Hirschsprung's Disease. JOURNAL OF CLINICAL RESEARCH, 2019, 36(2): 233-233.
[1] 冯杰雄.先天性巨结肠的诊断及治疗专家共识[J].中华小儿外科,2017,38(11):805-815. [2] Barlow-Anacker AJ.Immunostaining to visualize murine enteric nervous system development[J].J Vis Exp,2015,98:e52716. [3] Kawai H.Developmental markers of ganglion cells in the enteric nervous system and their application for evaluation of Hirschsprung disease[J].Pathol Int,2014,64(9):432-442. [4] Leonhard Bachmann.Immunohistochemical panel for the diagnosis of Hirschsprungs disease using antibodies to MAP2,calretinin,GLUT1 and S100[J].Histopathology,2015,66,824-835. [5] Szylberg L,Marszalek A.Diagnosis of Hirschsprung's disease with particular emphasis on histopathology.A systematic review of current literature[J].Prz Gastroenterol,2014,9(5):264-269. [6] Zani A,Eaton S,Morini F,et al.European paediatric surgeons' association survey on the management of Hirschsprung disease[J].Eur J Pediatr Surg,2017,27(1):96-101. [7] Sham MH,Lui VC,Fu M,et al.SOX10 is abnormally expressed Aganglionic bowel of Hirschsprung's disease infants[J].Gut,2001,49(2):220-226. [8] Bondurand N,Sham MH.The role of SOX10 during enteric nervous system development[J].Dev Biol,2013,382(1):330-343. [9] Sasselli V,Pachnis V,Burns AJ.The enteric nervous system[J].Dev Biol,2012,366(1):64-73. [10] Mwizerwa O, Das P, Nagy N,et al.Gdnf is mitogenic,neurotrophic,and Chemoa-ttractive to Enteric Neural Crest Cells in the Embryonic Colon[J].Dev Dyn,2011,240(6):1402-1411. [11] 胡宝辉,冯亮,邹音.先天性巨结肠不同节段肠壁神经、平滑肌的分布及临床意义[J].中华实验外科杂志,2018,25(9):1164-1166.
2019, Vol. 36 
