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Fibrosing colonopathy

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Medical condition
Fibrosing colonopathy
Other namesAbdominal pain, abdominal swelling, vomiting, constipation
CausesHigh doses of pancreatic enzyme supplements
Risk factorsYoung age, prior surgery of the intestines, certain medications including corticosteroids and H2 blockers
Frequency>60 cases reported

Fibrosing colonopathy is a disease that arises in people with cystic fibrosis treated with high doses of pancreatic enzyme supplements. Symptoms are non-specific with abdominal pain, abdominal swelling, vomiting, and constipation.

Risk factors include being young, prior surgery of the intestines, and the use of certain medications including corticosteroids and H2 blockers. It may appear similar to distal intestinal obstruction syndrome or inflammatory colitis such as Crohn's disease.

A maximum dose of 10,000 IU of lipase per kilogram per day is recommended for pancreatic enzyme supplementation to prevent this condition. More than 60 cases have been described as of 1999. The disease was suggested to be caused by methacrylic acid copolymer which is used as coating for delayed release of enzymes but there is no reliable evidence for that.

Signs and symptoms

Clinical symptoms of fibrosing colonopathy can be similar to those of intestinal obstruction and include constipation or diarrhea, abdominal pain, hematochezia, vomiting, nausea, and poor weight gain.

Causes

The precise etiopathogenesis is still unknown. Young age (2–13 years), gastrointestinal history (DIOS, meconium ileus), abdominal surgery in the past, HDPE, and use of histamine H2-receptor blockers, corticosteroids, or recombinant human deoxyribonuclease (DNase) are risk factors. Fibrosing colonopathy and cystic fibrosis have a strong correlation.

Diagnosis

The afflicted colon's histologic findings include thickening of the muscularis propria, submucosal fibrosis, a cobblestone-like appearance, and persistent mucosal inflammation. With relative rectal sparing, imaging can demonstrate diffuse narrowing, shortening, and loss of haustration of the colonic lumen.

See also

References

  1. ^ Smyth RL (May 1996). "Fibrosing colonopathy in cystic fibrosis". Archives of Disease in Childhood. 74 (5): 464–8. doi:10.1136/adc.74.5.464. PMC 1511547. PMID 8669970.
  2. ^ Lloyd-Still, JD; Beno, DW; Kimura, RM (June 1999). "Cystic fibrosis colonopathy". Current Gastroenterology Reports. 1 (3): 231–7. doi:10.1007/s11894-999-0040-4. PMID 10980955. S2CID 37595322.
  3. Schibli, S; Durie, PR; Tullis, ED (November 2002). "Proper usage of pancreatic enzymes". Current Opinion in Pulmonary Medicine. 8 (6): 542–6. doi:10.1097/00063198-200211000-00010. PMID 12394164.
  4. Borum, M. L.; Ginsberg, A. L. (1999). "Lack of evidence for fibrosing colonopathy by 5-ASA in humans". Digestive Diseases and Sciences. 44 (7): 1494. doi:10.1023/a:1026676407838. PMID 10489937. S2CID 34629882.
  5. Terheggen, G; Dieninghoff, D; Rietschel, E; Drebber, U; Kruis, W; Leifeld, L (2011). "Successful Non-Invasive treatment of stricturing fibrosing colonopathy in an adult patient". European Journal of Medical Research. 16 (9). Springer Science and Business Media LLC: 411–414. doi:10.1186/2047-783x-16-9-411. ISSN 2047-783X. PMC 3352147. PMID 22024442.
  6. ^ Fiore, Dominic; Sohini, Vidhur; Mileti, Elizabeth; Fiore, Nicholas (June 9, 2023). "Fibrosing Colonopathy Presenting in a Patient with Cystinosis". JPGN Reports. 4 (3). Wiley: e321. doi:10.1097/pg9.0000000000000321. ISSN 2691-171X. PMC 10435035. PMID 37600607.
  7. FitzSimmons, Stacey C.; Burkhart, Greg A.; Borowitz, Drucy; Grand, Richard J.; Hammerstrom, Thomas; Durie, Peter R.; Lloyd-Still, John D.; Lowenfels, Albert B. (1997). "High-Dose Pancreatic-Enzyme Supplements and Fibrosing Colonopathy in Children with Cystic Fibrosis". New England Journal of Medicine. 336 (18). Massachusetts Medical Society: 1283–1289. doi:10.1056/nejm199705013361803. ISSN 0028-4793.
  8. Pawel, Bruce R; de Chadarévian, Jean-Pierre; Franco, Maria E (1997). "The pathology of fibrosing colonopathy of cystic fibrosis: A study of 12 cases and review of the literature". Human Pathology. 28 (4). Elsevier BV: 395–399. doi:10.1016/s0046-8177(97)90025-3. ISSN 0046-8177. PMID 9104936.
  9. Schwarzenberg, S.J.; Wielinski, C.L.; Shamieh, I.; Carpenter, B.L.M.; Jessurun, J.; Weisdorf, S.A.; Warwick, W.J.; Sharp, H.L. (1995). "Cystic fibrosis–associated colitis and fibrosing colonopathy". The Journal of Pediatrics. 127 (4). Elsevier BV: 565–570. doi:10.1016/s0022-3476(95)70113-3. ISSN 0022-3476. PMID 7562277.

Further reading

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