Cystic fibrosis-associated colitis and fibrosing colonopathy

S. J. Schwarzenberg, C. L. Wielinski, I. Shamieh, B. L.M. Carpenter, J. Jessurun, S. A. Weisdorf, W. J. Warwick, H. L. Sharp

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Abstract

Objective: To describe our experience with cystic fibrosis (CF)-associated colitis and fibrosing colonopathy, and to assess treatment strategies. Study design: We reviewed hospital charts and autopsy reports of all University of Minnesota patients with CF between 1975 and August 1994. We identified six patients with colonopathy and compared them with a cohort of 79 patients with CF in the same age range and seen during the same period. Results: All patients with colonopathy had bloody diarrhea; five of the six had abdominal pain. Stool frequency and related symptoms distinguished the patients with colonopathy from the cohort population. All took a higher median dose of pancreatic enzymes than the cohort population during the 3 months preceding the onset of symptoms (p <0.002). For all six patients, barium studies revealed loss of haustration, and shortening and diffuse narrowing of the colonic lumen with relative rectal sparing. The distal ileal mucosa was irregular in four patients. A histopathologic study reveal fibrosis of the submucosa or lamina propria, and focal acute cryptitis in all six patients. Other features included ascites (2/6) and nodular regenerative hyperplasia of the liver (1/6). One patient continues to have symptoms, three had subtotal colectomy, and the condition of two improved after a regimen including a low-fat diet, withholding of pancreatic enzymes, and supplemental parenteral nutrition was initiated. Conclusions: Fibrosing colonopathy represents a newly recognized gastrointestinal complication of cystic fibrosis. Affected persons have taken larger doses of pancreatic enzymes than similar patients with cystic fibrosis, and have bloody diarrhea. We developed a medical protocol that may avoid surgical resection of the colon in some of these patients. (J PEDIATR 1995;127:565-70).

Original languageEnglish (US)
Pages (from-to)565-570
Number of pages6
JournalThe Journal of Pediatrics
Volume127
Issue number4
DOIs
StatePublished - Jan 1 1995

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Colitis
Cystic Fibrosis
Diarrhea
Mucous Membrane
Enzymes
Fat-Restricted Diet
Colectomy
Parenteral Nutrition
Barium
Ascites
Abdominal Pain
Population
Hyperplasia
Autopsy
Colon
Fibrosis

Cite this

Schwarzenberg, S. J., Wielinski, C. L., Shamieh, I., Carpenter, B. L. M., Jessurun, J., Weisdorf, S. A., ... Sharp, H. L. (1995). Cystic fibrosis-associated colitis and fibrosing colonopathy. The Journal of Pediatrics, 127(4), 565-570. https://doi.org/10.1016/S0022-3476(95)70113-3

Cystic fibrosis-associated colitis and fibrosing colonopathy. / Schwarzenberg, S. J.; Wielinski, C. L.; Shamieh, I.; Carpenter, B. L.M.; Jessurun, J.; Weisdorf, S. A.; Warwick, W. J.; Sharp, H. L.

In: The Journal of Pediatrics, Vol. 127, No. 4, 01.01.1995, p. 565-570.

Research output: Contribution to journalArticle

Schwarzenberg, SJ, Wielinski, CL, Shamieh, I, Carpenter, BLM, Jessurun, J, Weisdorf, SA, Warwick, WJ & Sharp, HL 1995, 'Cystic fibrosis-associated colitis and fibrosing colonopathy', The Journal of Pediatrics, vol. 127, no. 4, pp. 565-570. https://doi.org/10.1016/S0022-3476(95)70113-3
Schwarzenberg SJ, Wielinski CL, Shamieh I, Carpenter BLM, Jessurun J, Weisdorf SA et al. Cystic fibrosis-associated colitis and fibrosing colonopathy. The Journal of Pediatrics. 1995 Jan 1;127(4):565-570. https://doi.org/10.1016/S0022-3476(95)70113-3
Schwarzenberg, S. J. ; Wielinski, C. L. ; Shamieh, I. ; Carpenter, B. L.M. ; Jessurun, J. ; Weisdorf, S. A. ; Warwick, W. J. ; Sharp, H. L. / Cystic fibrosis-associated colitis and fibrosing colonopathy. In: The Journal of Pediatrics. 1995 ; Vol. 127, No. 4. pp. 565-570.
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AU - Jessurun, J.

AU - Weisdorf, S. A.

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AU - Sharp, H. L.

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N2 - Objective: To describe our experience with cystic fibrosis (CF)-associated colitis and fibrosing colonopathy, and to assess treatment strategies. Study design: We reviewed hospital charts and autopsy reports of all University of Minnesota patients with CF between 1975 and August 1994. We identified six patients with colonopathy and compared them with a cohort of 79 patients with CF in the same age range and seen during the same period. Results: All patients with colonopathy had bloody diarrhea; five of the six had abdominal pain. Stool frequency and related symptoms distinguished the patients with colonopathy from the cohort population. All took a higher median dose of pancreatic enzymes than the cohort population during the 3 months preceding the onset of symptoms (p <0.002). For all six patients, barium studies revealed loss of haustration, and shortening and diffuse narrowing of the colonic lumen with relative rectal sparing. The distal ileal mucosa was irregular in four patients. A histopathologic study reveal fibrosis of the submucosa or lamina propria, and focal acute cryptitis in all six patients. Other features included ascites (2/6) and nodular regenerative hyperplasia of the liver (1/6). One patient continues to have symptoms, three had subtotal colectomy, and the condition of two improved after a regimen including a low-fat diet, withholding of pancreatic enzymes, and supplemental parenteral nutrition was initiated. Conclusions: Fibrosing colonopathy represents a newly recognized gastrointestinal complication of cystic fibrosis. Affected persons have taken larger doses of pancreatic enzymes than similar patients with cystic fibrosis, and have bloody diarrhea. We developed a medical protocol that may avoid surgical resection of the colon in some of these patients. (J PEDIATR 1995;127:565-70).

AB - Objective: To describe our experience with cystic fibrosis (CF)-associated colitis and fibrosing colonopathy, and to assess treatment strategies. Study design: We reviewed hospital charts and autopsy reports of all University of Minnesota patients with CF between 1975 and August 1994. We identified six patients with colonopathy and compared them with a cohort of 79 patients with CF in the same age range and seen during the same period. Results: All patients with colonopathy had bloody diarrhea; five of the six had abdominal pain. Stool frequency and related symptoms distinguished the patients with colonopathy from the cohort population. All took a higher median dose of pancreatic enzymes than the cohort population during the 3 months preceding the onset of symptoms (p <0.002). For all six patients, barium studies revealed loss of haustration, and shortening and diffuse narrowing of the colonic lumen with relative rectal sparing. The distal ileal mucosa was irregular in four patients. A histopathologic study reveal fibrosis of the submucosa or lamina propria, and focal acute cryptitis in all six patients. Other features included ascites (2/6) and nodular regenerative hyperplasia of the liver (1/6). One patient continues to have symptoms, three had subtotal colectomy, and the condition of two improved after a regimen including a low-fat diet, withholding of pancreatic enzymes, and supplemental parenteral nutrition was initiated. Conclusions: Fibrosing colonopathy represents a newly recognized gastrointestinal complication of cystic fibrosis. Affected persons have taken larger doses of pancreatic enzymes than similar patients with cystic fibrosis, and have bloody diarrhea. We developed a medical protocol that may avoid surgical resection of the colon in some of these patients. (J PEDIATR 1995;127:565-70).

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