NSE和S-100在先天性胆管扩张症发病机制中的协同作用

The collaborative role of NSE and S-100 in the pathogenesis of congenital biliary dilatation

目的探讨神经细胞特异性烯醇化酶（NsE）和S-100蛋白在先天性胆管扩张症（CBD）发病中的作用及临床意义。方法2007-2009年连续CBD患儿36例，均行囊肿和胆囊切除、肝门空肠吻合术，切取囊肿远、近端囊壁和胆囊壁为实验组。选取非正常怀孕或因其母亲特殊原因需终止妊娠胎儿15例的肝外胆管和胆囊为对照组。每组标本分别固定、包埋，免疫组织化学染色检测对比其NSE和S-100表达，比较其染色结果和分布特点，并对囊肿直径和胆道不同部位NSE和S-100的表达进行相关分析。结果NSE和S-100在囊肿远端表达（1．86±1．29，1．81±1．04）均弱于囊肿近端表达（3．61±1．92，3．58±1．95）（P〈0．05）；囊肿远端与胆囊（3．42±1．99，3．72±2．08）间差异有统计学意义（P〈0．05），囊肿近端与胆囊间差异无统计学意义（P〉0．05）。胎儿胆管（4．86±2．97，5．14±2．73）和胎儿胆囊（3．71±2．14，4．00±1．63）间NSE和S-100差异无统计学意义（P〉0．05）。组间比较，囊肿远端与胎儿胆管、胆囊间NSE和S-100差异均有统计学意义（P〈0．05）；囊肿近端与胎儿胆管、胆囊问差异无统计学意义（P〈0．05）；两组胆囊间差异无统计学意义（P〉0．05）。囊肿直径和胆道不同部位NSE和S-100的表达均呈负相关（P〈0．05）。结论胆道远端神经节细胞和神经纤维的发育和分布受限与CBD发病关系密切，与其囊肿大小呈负相关；NSE与S-100表达在CBD发病中协同互补，可为CBD术中囊肿切除范围的正确判断提供理论依据。

Objective To explore the role and clinical significance of NSE and S-100 in the pathogenesis of congenital biliary dilatation （CBD）. Methods From 2007 to 2009, 36 children with CBD underwent choledochal cyst excision and hepaticojejunostomy. The proximal cyst walls, distal cysts walls and gallbladder walls were collected as experimental group. At the same time, the extrahepatic bile ducts and gallbladders of 15 fetus who were late terminated because of unplanned pregnancy were selected as the control group. Then the specimens of two groups were fixed, embedded, and immunohistochemical stained. In different parts of biliary tracts, the expressions and distributions of NSE and S-100 were compared and the relation were analyzed between the diameters of cysts and the expressions of NSE and S-100. The results were statistically analyzed by SPSS12. 0 for windows software. Results The expressions of NSE and ~100 in the distal cyst （1.86 ± 1.29, 1. 81 ±1.04） were weaker than those in the proximal ones （3. 61 ± 1.92, 3. 58 ± 1.95） （P〈0. 05）. The expressions of these two proteins in gallbladder （3. 42 ±1.99 vs 3. 72±2. 08） were also significant lower than those in the distal cyst （P〈0. 05）, but no difference could be noted between the proximal cyst and gallbladder. There were no significant difference of these two proteins between fetal bile duct（4. 86 ± 2. 97, 5. 14± 2. 73） and fetal gallbladder （3.71± 2. 14, 4.00 ±1.63） （P〈0.05） . The expressions of NSE and S-100 in distal cysts were also lower than those of fetal bile ducts and gallbladders（P〈0. 05）. The diameter of cysts and the expressions of NSE and S-100 in different parts of biliary tracts were all negatively correlated each other （P〈0. 05）. Conclusions The impaired distributions of ganglion cells and nerve fibers in the distal bile duct are related to the pathogenesis of CBD. The development of ganglion cells andnerve fibers are negatively related to the size of cysts, The collaborative role of NSE and S-100 can provide theoretical basis for the right range of cyst excision.