Neuropathy and vasculopathy in colonic strictures from children with cystic fibrosis

• Published in: Journal of pediatric surgery 1996-07, Vol.31 (7), p.945-950
• Main Authors: Collins, Margaret H, Azzarelli, Biagio, West, Karen W, Chong, Sonny K.F, Maguiness, Karen M, Stevens, John C
• Format: Article
• Language: English
• Subjects: Acetylcholinesterase - analysis; Adolescent; Adrenergic Fibers - pathology; Biological and medical sciences; Catecholamines - analysis; Child; Child, Preschool; Cholinergic Fibers - pathology; Choristoma - pathology; Colon - blood supply; Colon - innervation; Colon - pathology; Colonic Diseases - chemically induced; Colonic Diseases - pathology; Constriction, Pathologic - chemically induced; Constriction, Pathologic - pathology; Cystic Fibrosis - drug therapy; Cystic Fibrosis - pathology; Errors of metabolism; Female; Ganglia - pathology; Humans; Hyperplasia; Infant, Newborn; Intestinal Mucosa - blood supply; Intestinal Mucosa - innervation; Intestinal Mucosa - pathology; Male; Medical sciences; Mesenteric Arteries - pathology; Metabolic diseases; Miscellaneous hereditary metabolic disorders; Pancreas - enzymology; Pancreatic Extracts - adverse effects; Peripheral Nervous System Diseases - chemically induced; Peripheral Nervous System Diseases - pathology; Tunica Intima - pathology; Tyrosine 3-Monooxygenase - analysis; Vascular Diseases - chemically induced; Vascular Diseases - pathology; Vasoactive Intestinal Peptide - analysis
• ISSN: 0022-3468; 1531-5037
• 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.