Gong Z H, Xiao X, Chen L
Department of Pediatric Surgery, Shanghai Children's Hospital, Shanghai Jiaotong University, Shanghai, P. R. China.
Eur J Pediatr Surg. 2007 Feb;17(1):12-6. doi: 10.1055/s-2007-964950.
The study population was divided into 4 groups: 12 infants with choledochal cyst, aged 4 m to 12 m, were classified as the infant choledochal cyst (ICC) group; 36 children, aged 1 y to 14 y, were classified as the children with choledochal cyst (CCC) group; while 18 patients, aged 2 m to 5 m, with biliary atresia (BA) were included as positive controls; and 14 infants, aged 1 d to 3 y, who died from non-liver diseases served as negative controls (CON). Liver specimens were examined using H&E sections to score fibrosis by means of Ohkuma's classification, and immunohistochemical sections were evaluated by counting the cells positive for cytokeratin (CK) and human leukocyte antigen-DR (HLA-DR) to discover the pathogenic factors of fibrosis.
Most ICC patients had clinical biliary obstruction. The liver fibrosis score was highest in the BA group (2.9 +/- 0.7). The fibrosis score in the ICC group was higher (2.5 +/- 0.9) than that of the CCC (1.5 +/- 1.2; p < 0.05) and of the CON (0.1 +/- 0.4; p < 0.01) groups. The densities of CK-positive cells were 164 +/- 80/HP, 253 +/- 165/HP, 70 +/- 57/HP and 23 +/- 12/HP in the BA, ICC, CCC and CON groups, respectively, and differed significantly (p < 0.01) with the exception of the ICC vs. the BA group. The densities of HLA-DR positive cells were 130 +/- 72/HP, 98 +/- 54/HP, 96 +/- 50/HP and 36 +/- 13/HP in the portal area in the BA, ICC, CCC and CON groups, respectively. The density was lowest in the CON group (p < 0.01).
In patients with choledochal cyst, liver fibrosis is more common and severe in infants than in children. Obstruction of the bile duct and proliferation of bile duct cells were the main pathogenic factors for fibrosis, while HLA-DR mediating immuno-injury may play a limited role.
研究人群分为4组：12例年龄4个月至12个月的胆总管囊肿患儿被归为婴儿胆总管囊肿（ICC）组；36例年龄1岁至14岁的胆总管囊肿患儿被归为儿童胆总管囊肿（CCC）组；18例年龄2个月至5个月的胆道闭锁（BA）患儿作为阳性对照；14例年龄1天至3岁因非肝脏疾病死亡的婴儿作为阴性对照（CON）。肝组织标本用苏木精-伊红（H&E）切片，采用大隈分类法对纤维化进行评分，免疫组化切片通过计数细胞角蛋白（CK）和人类白细胞抗原-DR（HLA-DR）阳性细胞来发现纤维化的致病因素。
大多数ICC患者有临床胆道梗阻。BA组肝纤维化评分最高（2.9±0.7）。ICC组纤维化评分（2.5±0.9）高于CCC组（1.5±1.2；p<0.05）和CON组（0.1±0.4；p<0.01）。BA组、ICC组、CCC组和CON组CK阳性细胞密度分别为164±80/HP、253±165/HP、70±57/HP和23±12/HP，除ICC组与BA组外差异有统计学意义（p<0.01）。BA组、ICC组、CCC组和CON组门静脉区HLA-DR阳性细胞密度分别为130±72/HP、98±54/HP、96±50/HP和36±13/HP。CON组密度最低（p<0.01）。
胆总管囊肿患者中，婴儿肝纤维化比儿童更常见且更严重。胆管梗阻和胆管细胞增殖是纤维化的主要致病因素，而介导免疫损伤的HLA-DR可能起有限作用。
