Fibrosing colonopathy
Other namesAbdominal pain, abdominal swelling, vomiting, constipation[1]
CausesHigh doses of pancreatic enzyme supplements[1]
Risk factorsYoung age, prior surgery of the intestines, certain medications including corticosteroids and H2 blockers[2]
Frequency>60 cases reported[2]

Fibrosing colonopathy is a disease that arises in people with cystic fibrosis treated with high doses of pancreatic enzyme supplements.[1][2] Symptoms are non-specific with abdominal pain, abdominal swelling, vomiting, and constipation.[1]

Risk factors include being young, prior surgery of the intestines, and the use of certain medications including corticosteroids and H2 blockers.[2] It may appear similar to distal intestinal obstruction syndrome or inflammatory colitis such as Crohn's disease.[1]

A maximum dose of 10,000 IU of lipase per kilogram per day is recommended for pancreatic enzyme supplementation to prevent this condition.[3] More than 60 cases have been described as of 1999.[2] 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.[4]

Signs and symptoms

Clinical symptoms of fibrosing colonopathy can be similar to those of intestinal obstruction and include constipation or diarrhea, abdominal pain, hematochezia,[5] vomiting, nausea, and poor weight gain.[6]

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.[7] Fibrosing colonopathy and cystic fibrosis have a strong correlation.[6]

Diagnosis

The afflicted colon's histologic findings include thickening of the muscularis propria, submucosal fibrosis, a cobblestone-like appearance, and persistent mucosal inflammation.[8] With relative rectal sparing, imaging can demonstrate diffuse narrowing, shortening, and loss of haustration of the colonic lumen.[9]

See also

References

  1. 1 2 3 4 5 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. 1 2 3 4 5 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. Springer Science and Business Media LLC. 16 (9): 411–414. doi:10.1186/2047-783x-16-9-411. ISSN 2047-783X. PMC 3352147. PMID 22024442.
  6. 1 2 Fiore, Dominic; Sohini, Vidhur; Mileti, Elizabeth; Fiore, Nicholas (June 9, 2023). "Fibrosing Colonopathy Presenting in a Patient with Cystinosis". JPGN Reports. Wiley. 4 (3): 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. Massachusetts Medical Society. 336 (18): 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. Elsevier BV. 28 (4): 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. Elsevier BV. 127 (4): 565–570. doi:10.1016/s0022-3476(95)70113-3. ISSN 0022-3476. PMID 7562277.

Further reading

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