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Fibrosing colonopathy in cystic fibrosis: results of a case-control study
Insights
High-strength pancreatic enzyme use is linked to fibrosing colonopathy in cystic fibrosis (CF) patients. This dose-related association suggests careful enzyme management is crucial for CF care.
Area of Science:
- Gastroenterology
- Pediatrics
- Genetics
Background:
- Fibrosing colonopathy (FC) is a rare but serious complication in children with cystic fibrosis (CF).
- First described in 1994, FC's etiology remained unclear, prompting investigation into potential contributing factors.
Purpose of the Study:
- To investigate associations between fibrosing colonopathy and treatments in pediatric cystic fibrosis patients.
- To identify specific risk factors, particularly related to pancreatic enzyme replacement therapy and laxative use.
Main Methods:
- A nested case-control study was conducted within the UK CF population.
- 14 FC cases were matched with four controls each, using data from case records and family interviews.
- Statistical analysis focused on the 12 months preceding surgery, examining enzyme preparation strength, protease use, and laxative intake.
Main Results:
- A significant dose-related association was found between high-strength pancreatic enzyme preparations and FC occurrence.
- Increased odds ratios were observed for high-strength capsules (OR 1.45) and protease use (OR 1.55).
- Laxative use was also independently predictive of FC (OR 2.42).
Conclusions:
- High-strength pancreatic enzyme preparations are associated with an increased risk of fibrosing colonopathy in CF patients.
- The risk appears to be dose-dependent, highlighting the importance of appropriate enzyme dosing.
- Laxative use is an independent risk factor, suggesting comprehensive management strategies are needed.
Abstract:
Fibrosing colonopathy was first described in cystic fibrosis (CF) children in 1994. We have done a nested case-control study to identify possible associations with this condition. A case ascertainment within the UK CF population to identify any cases that occurred between January, 1984, and April, 1994, found 14 cases, all under 14 years and confirmed by independent histopathological review. All had presented since April, 1993; 12 were boys and six had received some or all of their care in Liverpool. Each case was matched, by date of birth, with four controls from the UK CF Registry. Information was obtained about cases and controls from their case records and by a structured interview with the families. In the 12 months before surgery, there was an association between the occurrence of fibrosing colonopathy and use of high-strength pancreatic enzyme preparations. This association was dose related. Odds ratio per extra 1000 high-strength capsules was 1.45 (95% CI 1.14-1.84). For use of protease, the odds ratio per million extra units per kg was 1.55 (1.19-2.03). For usage of individual high-strength products at any time during the 12 months before surgery some differences were observed; for Creon 25000 the odds ratio was 0.38 (0.10-1.42), for Nutrizym 22 43.4 (2.51-751), and for Pancrease HL 8.4 (1.95-36.1). These last two confidence intervals are extremely wide and compatible with these two products having the same odds ratios. Laxative use was independently predictive (odds ratio 2.42 [1.20-4.94]). We conclude that there is a dose-related association between high-strength pancreatic enzyme preparations and fibrosing colonopathy.