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Fibrosing colonopathy in cystic fibrosis
1Respiratory Unit, Royal Liverpool Children's Hospital.
Insights
Enteric coated pancreatic enzyme supplements improved cystic fibrosis care. However, high doses were linked to fibrosing colonopathy, a complication now seemingly prevented by dose adjustments.
Area of Science:
- Gastroenterology
- Pediatrics
- Pulmonology
Background:
- Enteric coated pancreatic enzyme supplements revolutionized cystic fibrosis care starting in the 1980s.
- Product refinements aimed to enhance patient compliance and acceptability.
- The unexpected emergence of fibrosing colonopathy complicated enzyme therapy.
Purpose of the Study:
- To review the impact of pancreatic enzyme supplements in cystic fibrosis care.
- To address the complication of fibrosing colonopathy associated with these supplements.
- To evaluate the trend in fibrosing colonopathy cases following dosage adjustments.
Main Methods:
- Review of clinical observations and case reports regarding pancreatic enzyme supplements.
- Analysis of trends in fibrosing colonopathy incidence in cystic fibrosis patients.
- Correlation of enzyme preparation strength with adverse event occurrences.
Main Results:
- High-strength pancreatic enzyme preparations were associated with fibrosing colonopathy.
- A significant decrease in histologically confirmed cases of fibrosing colonopathy was observed since July 1994 in the UK.
- No cases were reported in children on high-strength preparations after July 1994.
Conclusions:
- The principle of 'More is not necessarily better' applies to pancreatic enzyme products.
- Careful dosing and formulation of pancreatic enzymes are crucial for preventing fibrosing colonopathy.
- Continued monitoring and research are needed to fully understand and prevent this complication.
Abstract:
The introduction of enteric coated pancreatic enzyme supplements in the early 1980s was undoubtedly one of the major advances in the care of children with cystic fibrosis. Further refinements in the presentation of these preparations inevitably followed, to improve patient acceptability and compliance. The emergence of fibrosing colonopathy took clinicians dealing with cystic fibrosis completely by surprise, and in the last two years there has been a gradual appreciation that as far as pancreatic enzyme products are concerned 'More is not necessarily better'. However, it is encouraging that, in the UK, there have been no histologically confirmed cases in children receiving high strength pancreatic enzyme preparations since July 1994. Hopefully this trend will continue and the causal factors will be defined, ensuring that this serious complication can be effectively prevented in the future.