| Fibrosing colonopathy | |
|---|---|
| Other names | Abdominal pain, abdominal swelling, vomiting, constipation [1] |
| Causes | High doses of pancreatic enzyme supplements [1] |
| Risk factors | Young 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]
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]
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]
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]