Case Report

Fibrosing Colonopathy in an Adult Cystic Fibrosis Patient After Discontinuing Pancreatic Enzyme Therapy

Authors: Elizabeth H. Mack, MD, Allan S. Brett, MD, Daniel Brown, MD

Abstract

Fibrosing colonopathy, a complication of cystic fibrosis, has generally been reported in young children with exposure to high doses of pancreatic enzymes. The authors report the case of a 25-year-old male with cystic fibrosis who presented with gradually progressive symptoms of bowel obstruction. Pathologic examination of the right colon revealed findings consistent with fibrosing colonopathy. This case is distinctive because of the adult presentation, and because the patient's symptoms developed long after he had discontinued taking a high-strength enzyme preparation. This case suggests that multiple etiologic factors, and not simply exposure to pancreatic enzymes, may result in fibrosing colonopathy in adults with cystic fibrosis.


Key Points


* Fibrosing colonopathy is a complication of cystic fibrosis that has been diagnosed almost exclusively in young children.


* Exposure to high doses of pancreatic enzymes is frequently regarded as the cause of fibrosing colonopathy.


* In this case, a 25-year-old man with cystic fibrosis developed symptoms and pathologic findings consistent with fibrosing colonopathy, long after stopping pancreatic enzyme therapy.


* Multiple etiologic factors, and not simply exposure to pancreatic enzymes, may result in fibrosing colonopathy in adults with cystic fibrosis.

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References

1. Reichard KW, Vinocur CD, Franco M, et al. Fibrosing colonopathy in children with cystic fibrosis. J Pediatr Surg 1997;32:237–242.
 
2. Schwarzenburg SJ, Wielinski CL, Shamleh I, et al. Cystic fibrosis-associated colitis and fibrosing colonopathy. J Pediatr 1995;127:565–570.
 
3. Powell CJ. Colonic toxicity from pancreatins: a contemporary safety issue. Lancet 1999;353:911–915.
 
4. Dodge JA. The aetiology of fibrosing colonopathy. Postgrad Med J 1996;72(suppl 2):S52–S55.
 
5. FitzSimmons SC, Burkhart GA, Borowitz D, et al. High-dose pancreatic-enzyme supplements and fibrosing colonopathy in children with cystic fibrosis. New Engl J Med 1997;336:1283–1289.
 
6. The Cystic Fibrosis Genotype-Phenotype Consortium. Correlation between genotype and phenotype in patients with cystic fibrosis. New Engl J Med 1993;329:1308–1313.
 
7. Borowitz DS, Grand RJ, Durie PR. Use of pancreatic enzyme supplements for patients with cystic fibrosis in the context of fibrosing colonopathy. J Pediatr 1995;127:681–684.
 
8. Smyth RL, van Velzen D, Smyth AR, et al. Strictures of ascending colon in cystic fibrosis and high-strength pancreatic enzymes. Lancet 1994;343:85–86.
 
9. Oades PJ, Bush A, Ong PS, et al. High-strength pancreatic enzyme supplementation and large bowel strictures in cystic fibrosis [letter]. Lancet 1994;343:109.
 
10. Campbell CA, Forrest J, Musgrove C. High strength pancreatic enzyme supplementation and large bowel strictures in cystic fibrosis [letter]. Lancet 1994;343:109–110.
 
11. van Velzen D, Ball LM, Dezfulian AR, et al. Comparative and experimental pathology of fibrosing colonopathy. Postgrad Med J 1996;72(suppl 2):S39–S48.
 
12. Frieman JP, FitzSimmons SC. Colonic strictures in patients with cystic fibrosis: results of a survey of 114 cystic fibrosis centers in the United States. J Pediatr Gastroenterol Nutr 1996;22:153–156.
 
13. Bansi DS, Price A, Russell C, et al. Fibrosing colonopathy in an adult owing to over use of pancreatic enzyme supplements. Gut 2000;46:283–285.
 
14. Waters BL. Cystic fibrosis with fibrosing colonopathy in the absence of pancreatic enzymes.Pediatric and Developmental Pathology 1998;1:74–78.
 
15. Serban DE, Florescu P, Miu N. Fibrosing colonopathy revealing cystic fibrosis in a neonate before any pancreatic enzyme supplementation. J Pediatr Gastroenterol Nutr 2002;35:356–359.
 
16. Hausler M, Meilicke R, Biesterfeld S, et al. First adult patient with fibrosing colonopathy. Am J Gastroenterol 1998;93:1171–1172.
 
17. King SJ, van Velzen D, Smyth RL, et al. Strictures of the colon in cystic fibrosis. Clinical Radiology1994;49:476–477.
 
18. Haber HP, Benda N, Fitzke G, et al. Colonic wall thickness measured by ultrasound: striking differences in patients with cystic fibrosis versus healthy controls. Gut 1997;40:406–411.