Collins M H, Azzarelli B, West K W, Chong S K, Maguiness K M, Stevens J C
Division of Pediatric Pathology, Indiana University School of Medicine, Riley Hospital for Children, Indianapolis, USA.
J Pediatr Surg. 1996 Jul;31(7):945-50. doi: 10.1016/s0022-3468(96)90418-2.
Colonic strictures are rare in patients who have cystic fibrosis, but recently have developed in those who have been treated with delayed-release high-dose pancreatic enzyme supplements. Colonic strictures from eight such pediatric patients showed neural abnormalities consisting of ganglion cell hyperplasia and ectopia, and intermyenteric plexus hyperplasia. Cholinergic and adrenergic stains of mucosal nerve fibers were more prominent in histological sections of the cystic fibrosis strictures than in sections from colons of children without cystic fibrosis. The mean grade of staining with acetylcholinesterase in the lamina propria of the strictured cystic fibrosis colons was 2.38 +/- 1.25, compared with .93 +/- .93 (P < .055) in bowels from children without cystic fibrosis. The mean grade for tyrosine hydroxylase staining in the lamina propria was 2 +/- .97 in the strictures and was .79 +/- .81 (P < .05) in the bowels of children who did not have cystic fibrosis. Vasoactive intestinal peptide staining in bowels from children with cystic fibrosis with and without stricture did not differ significantly from that of children without cystic fibrosis. Vasculopathy consisting of fibrointimal hyperplasia in submucosal veins and mesenteric arteries was found only in colonic strictures owing to cystic fibrosis. Colonic strictures in patients with cystic fibrosis who received high-dose pancreatic enzyme supplements contain ganglion cell abnormalities, and mucosal cholinergic and adrenergic activity may be increased in these strictures. The stricture vasculopathy may be drug-related and/or related to increased catecholamine activity.
结肠狭窄在囊性纤维化患者中较为罕见，但近期在接受缓释高剂量胰酶补充剂治疗的患者中出现。8例此类儿科患者的结肠狭窄显示出神经异常，包括神经节细胞增生和异位以及肌间神经丛增生。与无囊性纤维化儿童的结肠切片相比，囊性纤维化狭窄的组织学切片中黏膜神经纤维的胆碱能和肾上腺素能染色更为明显。囊性纤维化狭窄结肠固有层中乙酰胆碱酯酶染色的平均等级为2.38±1.25，而无囊性纤维化儿童肠道中的为0.93±0.93（P<0.055）。固有层中酪氨酸羟化酶染色的平均等级在狭窄处为2±0.97，在无囊性纤维化儿童的肠道中为0.79±0.81（P<0.05）。有或无狭窄的囊性纤维化儿童肠道中的血管活性肠肽染色与无囊性纤维化儿童的相比无显著差异。仅在囊性纤维化所致的结肠狭窄中发现由黏膜下静脉和肠系膜动脉纤维内膜增生组成的血管病变。接受高剂量胰酶补充剂的囊性纤维化患者的结肠狭窄含有神经节细胞异常，且这些狭窄处的黏膜胆碱能和肾上腺素能活性可能增加。狭窄血管病变可能与药物相关和/或与儿茶酚胺活性增加有关。
