肠神经节细胞未成熟:生理与病理现象的探讨

Characteristics of intestinal immature ganglion cell in physiological and pathological conditions

目的 探讨生理与病理性节细胞未成熟的本质区别 ,以确切诊断节细胞未成熟症(IGC)。方法 ①选取HD术后恢复顺利 2 9例和合并肠炎 37例 ,单纯类缘病 (HAD)术后恢复顺利者2 6例的近端结肠 ,对照 2 2例的横结肠。年龄 18d～ 4岁。石蜡切片HE染色 ,光镜下统计各切面含成熟节细胞肌间神经丛的分布。②选取 12例HD术后恢复好的病变段、近端 (年龄 5个月～ 2岁 ) ,节细胞减少症 (年龄 7个月～ 3.5岁 )和IGC(年龄 2～ 4岁 )的病变段各 3例的结肠 ,2 1例对照的横结肠。分别行突触素 (SY)和组织蛋白酶D(CathepsinD)免疫组化染色。光镜下观察肠壁肌层SY和节细胞胞浆CathepsinD免疫活性的分布。 结果 功能正常结肠壁普遍存在未成熟节细胞 ,类似IGC病理特征的“节细胞未成熟现象”较普遍 ;肠壁总体节细胞中成熟与未成熟的比例与年龄不密切正相关。所有实验对象环行肌层SY免疫活性表达较一致。对照组和HD近端正常肠壁 ,环行肌SY免疫活性普遍丰富 ;HD和节细胞减少症病变段SY免疫活性均不同程度减低 ;IGC病变段表现丰富。功能正常结肠壁均见CathepsinD呈成熟和未成熟形态表达的节细胞 ;“节细胞未成熟现象”见一定数量成熟形态的节细胞 ,而IGC难于见到 ;HD病变段CathepsinD呈阴性表达。 结论 维持消化道正常动力 ,只?

Objective To explore a reliable method of identifying immaturity of ganglion cells (IGC) by investigating difference of immature ganglion cell between the physiological and pathological conditions. Methods Full thickness bowel specimens were obtained from the proximal margins of the resected colons of HD patients (29 no complications and 37 enterocolitis postoperatively), isolated dysganglionosis (26 no complications postoperatively), and the transverse colons of 22 controls. Their ages ranged from 18 days to 4 years. The number of myenteric plexuses containing mature ganglion cell in HE staining sections were counted under light microscope. Full thickness specimens obtained from 12 aganglionic and their proximal segments (age range 2 to 4 years), 3 diseased segments of hypoganglionosis (age range 7 months to 3.5 years) and IGC (age range 5 months to 2 years), and from 21 controls were examined, using SY and Cathepsin D immunohistochemistry respectively. The immunoreactivity of SY in muscular layers and that of Cathepsin D in ganglion-cell were observed under light microscope. Results In HE staining, not all the ganglion cells showed mature morphologically in the normal colonic walls, and the ratio between the mature and immature ganglion cells was not correlated to the age. Each group contained some cases similar to IGC morphologically, in which the mature ganglion cell is hardly observed. SY immunoreactive nerve fibers were abundant in the circular muscle layer of IGC, of the controls and of the proximal segments of HD. But it was markedly decreased in HD and hypoganglionosis. Cathepsin D immunoreactivity in the ganglion cells in normal bowel walls was detected in both the mature and immature staining patterns. However, it was not detected in the aganglionic segments. Ganglion cell in mature pattern was rarely observed in IGC. Conclusions A proper number of ganglion cells, not necessarily all, that developed mature in a bowel wall seems enough to maintain a normal gastrointestinal motility. A possible pathogenesis of IGC may be because the quantity of mature ganglion cell of the involved colon is too low to a certain extent. Quantitative analysis of mature ganglion cells in a bowel wall should be a more reliable method to diagnose IGC and to evaluate its motility function.