Calretinin, -Tubulin Immunohistochemistry, and Submucosal Nerve Trunks Morphology in Hirschsprung Disease: Possible Applications in Clinical Practice
Calretinin, -Tubulin Immunohistochemistry, and Submucosal Nerve Trunks Morphology in Hirschsprung Disease: Possible Applications in Clinical Practice
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DOI：
10.1097/mpg.0b013e3182a934c7
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发表时间：
2013-12
影响因子：
2.9
通讯作者：
Andrea Volpe;R. Alaggio;P. Midrio;L. Iaria;P. Gamba
中科院分区：
文献类型：
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作者：
Andrea Volpe;R. Alaggio;P. Midrio;L. Iaria;P. Gamba
目的：本研究的目的是探讨钙视网膜蛋白和微管蛋白的免疫组织化学表达与粘膜下神经干形态学在不同神经支配段的先天性巨结肠症（HD）和全结肠无神经节细胞症（TCA）。方法：对25例HD患者（22例HD，3例TCA）和18例正常对照者进行Calretinin和β-tubulin免疫组化检测。代表远端无神经节，过渡，和近端神经节段的部分进行了评价的视觉分级评分，微管蛋白也进行了评价，通过图像分析。组织形态学观察粘膜下神经干肥大和增生。近段长度与术后肠功能相关。结果：对照组显示强烈的钙视网膜蛋白和微管蛋白染色。在HD和TCA中，calretinin染色与神经节细胞的存在相关：远端为阴性，过渡段为微弱，近端为强。- 微管蛋白染色在HD的所有节段中均较弱，在TCA中为阴性。神经干以无节段为特征性增生和肥大，过渡段和节段神经体积逐渐减小。59%的HD患者存在一过性术后便秘、污染或小肠结肠炎，与近端节段长度或存在肥大神经干无明确关系。结论：钙视网膜蛋白是神经节细胞存在的可靠标志物，并且与神经肥大一起，它有助于识别过渡区。切除标本近段肠神经的长度和大小对术后肠功能无明显影响。微管蛋白表达沿着整个结肠道，包括近端神经节段减少，可能代表肠神经传递的潜在损害因素。
Objectives: The aim of this study was to investigate calretinin and -tubulin immunohistochemical expression together with submucosal nerve trunks morphology in differently innervated segments of Hirschsprung disease (HD) and total colonic aganglionosis (TCA). Methods: A total of 25 cases (22 HD, 3 TCA) and 18 controls were processed for calretinin and -tubulin immunohistochemistry. Sections representative of distal aganglionic, transition, and proximal ganglionic segments were evaluated by a visual grading score; -tubulin was evaluated also by image analysis. Submucosal nerve trunks hypertrophy and hyperplasia were measured by citomorphology. The length of proximal segment was correlated to postoperative bowel function. Results: Controls showed intense calretinin and -tubulin staining. In HD and TCA, calretinin staining was related to the presence of ganglion cells: negative in distal, faint in transition, intense in proximal segment. -Tubulin staining was weak in all of the segments of HD and negative in TCA. Hypertrophic and hyperplastic nerve trunks characterized aganglionic segment, and progressively decreasing nerve size was observed in transition and ganglionic segments. Transient postoperative constipation, soiling, or enterocolitis was present in 59% of patients with HD without clear relation to proximal segment length or presence of hypertrophic nerve trunks. Conclusions: Calretinin is a reliable marker of the presence of ganglion cells, and, together with nerve hypertrophy, it helps to identify the transition zone. Length and nerve size of proximal segment in resected specimen did not affect the postsurgical intestinal function. Reduced -tubulin expression along the entire colonic tract, included proximal ganglionic segments, may represent a potential impairing factor for the enteric neural transmission.