Colonic strictures in cystic fibrosis.

Green, M R; Southern, K.W.; Wolfe, Susan; Littlewood, J M; Najmaldin, Atousa; Wyatt, J I

doi:10.1136/adc.72.2.191

Cited by 11 publications

(2 citation statements)

References 3 publications

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“…There was, however, a tendency towards increased thickness in the distal ascending colon in the group of patients previously exposed to copolymer (fig 4), although this was largely caused by measurements in one patient who may have had an early colonic stricture. She presented at 7 years old with persistent abdominal pain and a mass in the right iliac fossa 10. A contrast examination showed loss of haustration and poor distensibility of the ascending colon (fig 5).…”

Section: Resultsmentioning

confidence: 99%

Colonic wall thickness, pancreatic enzyme dose and type of preparation in cystic fibrosis

Ramsden

Moya

Littlewood

1998

Archives of Disease in Childhood

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Increased colonic wall thickness has been reported in patients exposed to large doses of high strength pancreatic enzyme preparations who did not develop fibrosing colonopathy. This has been interpreted as evidence for a spectrum of subclinical disease. The relation between sonographically measured colonic wall thickness and pancreatic enzyme preparation and dose was studied in 86 children with cystic fibrosis (CF). Colonic wall thickness of a control group was also measured. The average thickness in all colonic regions was higher in the CF group (overall average range 0.7-2.5 mm v 0.6-1.4 mm in the control group). There was no significant relation between colonic wall thickness and age, sex, total dose of lipase, or copolymer. Apart from one patient with an early colonic stricture, none of those exposed to high doses of lipase, or the methacrylic acid copolymer Eudragit L30 D55, showed evidence of subclinical damage to the colon. The reproducibility of the sonographic measurements was poor. (Arch Dis Child 1998;79:339-343)

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Section: Resultsmentioning

confidence: 99%

Colonic wall thickness, pancreatic enzyme dose and type of preparation in cystic fibrosis

Ramsden

Moya

Littlewood

1998

Archives of Disease in Childhood

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“…In January 1994 Smyth et al published the initial description of fibrosing colonopathy (FC) in patients with CF whose only change in therapy was the prescription of very large doses of high‐strength pancreatic enzyme preparations (1). Subsequent reports have supported these findings(2‐13).…”

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confidence: 73%

Pancreatic Enzyme Supplementation in Cystic Fibrosis Patients Before and After Fibrosing Colonopathy

Stevens,

Maguiness,

Hollingsworth

et al. 1998

J. pediatr. gastroenterol. nutr.

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Background:In 1994 we cared for nine cystic fibrosis patients with fibrosing colonopathy. To evaluate the relationship between fibrosing colonopathy and supplemental pancreatic enzymes we reviewed our dosing of enzymes prior to fibrosing colonopathy development and then evaluated the subsequent effect of drastically reducing pancreatic enzyme dose.Methods:We retrospectively reviewed pancreatic enzyme dosing for 267 cystic fibrosis patients with pancreatic insufficiency. The supplemental enzyme history of nine patients with fibrosing colonopathy was contrasted with the history of 258 nonaffected patients. The pancreatic enzyme doses of 75 patients taking at least 6,000 U lipase/kg/meal were systematically reduced to approximately 2,000 lipase units/kg/meal. We evaluated the effect of this dose reduction on change in height and weight z scores one year after achievement of stable enzyme dose.Results:In the year prior to diagnosis patients with fibrosing colonopathy took a significantly larger pancreatic enzyme dose, whether assessed by highest dose or cumulative dose, than did nonaffected patients. Similar results were observed after controlling for sex and age. All 75 patients on at least 6,000 U lipase/kg/meal were able to tolerate a significant reduction in dose while achieving clinically acceptable nutrient absorption, with no change over one year in height and weight z scores.Conclusions:Our data demonstrate a strong relationship between very high doses of pancreatic enzyme supplementation and formation of fibrosing colonopathy. These very high doses do not appear to be needed for adequate nutrient absorption and growth.

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Neuropathy and vasculopathy in colonic strictures from children with cystic fibrosis

Collins

Azzarelli

West

et al. 1996

Journal of Pediatric Surgery

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Colonic strictures in cystic fibrosis.

Cited by 11 publications

References 3 publications

Colonic wall thickness, pancreatic enzyme dose and type of preparation in cystic fibrosis

Colonic wall thickness, pancreatic enzyme dose and type of preparation in cystic fibrosis

Pancreatic Enzyme Supplementation in Cystic Fibrosis Patients Before and After Fibrosing Colonopathy

Neuropathy and vasculopathy in colonic strictures from children with cystic fibrosis

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