title: Relation of thickening of colon wall to pancreatic-enzyme treatment in cystic fibrosis.
abstract: Fibrotic strictures of the colon in association with colon-wall thickening on abdominal ultrasound have been described in children with cystic fibrosis. We determined the prevalence of colon-wall thickening in 99 children with cystic fibrosis (aged 6 months to 17 years, 45 male) and 38 healthy controls (aged 1 month to 39 years, 21 male). In controls, peristalsis was uniformly rapid and maximum wall thickness of the fully relaxed colon was 0.8 mm (median 0.6 mm). 24% of the patients had a colon-wall thickness of 1.5 mm or more (maximum 3.4 mm); 48% of children on high-strength pancreatin receiving more than 265 units/kg per day of protease had thickening of more than 1.5 mm compared with less than 12% of children on any other dose. Children on high-strength pancreatin were 5.2 (95% CI 1.2-21) times more likely to have colon-wall thickness of 1.5 mm or more than children taking less than 265 units/kg per day of protease in a standard-strength preparation. This risk rose to 10 (2.5-39) times more likely if laxatives were taken with a high-strength preparation. Lipase or amylase intake, age, sex, genotype, and other medical or drug history were not significantly associated with colon-wall thickening. 1 child required a hemicolectomy for bowel obstruction and another developed haemorrhagic colitis. In 17 children, 2 months after stopping high-strength, high protease, preparations and efforts to reduce enzyme intake, colon-wall thickness had regressed from a median 2.0 mm (range 1.0-3.4) to 1.8 mm (0.9-2.7) (p = 0.005). The use of high-strength pancreatin preparations in conjunction with a high protease intake probably causes thickening of the wall of the colon.
authors.affiliation: Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, London, UK.
authors.forename: E J
authors.initials: EJ
authors.lastname: Mac Sweeney
authors.forename: P J
authors.initials: PJ
authors.lastname: Oades
authors.forename: R
authors.initials: R
authors.lastname: Buchdahl
authors.forename: M
authors.initials: M
authors.lastname: Rosenthal
authors.forename: A
authors.initials: A
authors.lastname: Bush
mesh_terms: D000293:Adolescent; D000328:Adult; D000681:Amylases / Q000008:administration & dosage / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003106:Colon / Q000000981:diagnostic imaging / Q000187:drug effects / Q000473:pathology*; D003550:Cystic Fibrosis / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000473:pathology; D010450:Endopeptidases / Q000008:administration & dosage / Q000627:therapeutic use*; D005355:Fibrosis; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008049:Lipase / Q000008:administration & dosage / Q000627:therapeutic use*; D016015:Logistic Models; D008297:Male; D012189:Retrospective Studies; D014463:Ultrasonography
chemical_list: D008049:Lipase; D000681:Amylases; D010450:Endopeptidases