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Fibrosing colonopathy

Field	Value
synonyms	Abdominal pain, abdominal swelling, vomiting, constipation
causes	High doses of pancreatic enzyme supplements
risks	Young 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.

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.

References

Smyth RL. (May 1996). "Fibrosing colonopathy in cystic fibrosis". [[Archives of Disease in Childhood]].
(June 1999). "Cystic fibrosis colonopathy.". Current Gastroenterology Reports.
(November 2002). "Proper usage of pancreatic enzymes.". Current Opinion in Pulmonary Medicine.
(1999). "Lack of evidence for fibrosing colonopathy by 5-ASA in humans". Digestive Diseases and Sciences.
(2011). "Successful Non-Invasive treatment of stricturing fibrosing colonopathy in an adult patient". Springer Science and Business Media LLC.
(June 9, 2023). "Fibrosing Colonopathy Presenting in a Patient with Cystinosis". Wiley.
(1997). "High-Dose Pancreatic-Enzyme Supplements and Fibrosing Colonopathy in Children with Cystic Fibrosis". Massachusetts Medical Society.
(1997). "The pathology of fibrosing colonopathy of cystic fibrosis: A study of 12 cases and review of the literature". Elsevier BV.
(1995). "Cystic fibrosis–associated colitis and fibrosing colonopathy". Elsevier BV.

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This article was imported from Wikipedia and is available under the Creative Commons Attribution-ShareAlike 4.0 License. Content has been adapted to SurfDoc format. Original contributors can be found on the article history page.

gastrointestinal-tract-disorders cystic-fibrosis

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