CFDB - Cystic Fibrosis DataBase

Cochrane Database of Systematic Reviews - Cochrane Review

Interventions for preventing distal intestinal obstruction syndrome (DIOS) in cystic fibrosis

Study design (if review, criteria of inclusion for studies)

Randomised-controlled trials (RCT) and quasi-RCTs. Cross-over trials considered for possible inclusion on an individual basis

List of included studies (1)



Children, young people and adults with cystic fibrosis


Pancreatic enzyme therapy

Outcome measures

Episodes of acute DIOS, presence of abdominal mass and abdominal pain

Main results

There was one trial with 20 participants (16 females) included in the review. The mean age of participants was 13.1 years. The trial was a double‐blinded, randomised cross‐over trial which had a duration of 12 months in total and compared high‐dose and low‐dose pancreatic enzyme therapy. As only the abstract of the trial was available, the overall risk of bias was judged to be unclear. The trial did not address either of our primary outcomes (time until resolution of DIOS and treatment failure rate), but reported episodes of acute DIOS, presence of abdominal mass and abdominal pain. There were no numerical data available for these outcomes, but the authors stated that there was no difference between treatment with high‐dose or low‐dose pancreatic enzymes. The overall certainty of the evidence was found to be very low.

Authors' conclusions

There is a clear lack of evidence for the treatment of DIOS in people with cystic fibrosis. The included abstract did not address our primary outcome measures and did not provide numerical data for the two secondary outcomes it did address. Therefore, we cannot justify the use of high‐dose pancreatic enzymes for treating DIOS, nor can we comment on the efficacy and safety of other laxative agents. From our findings, it is clear that more randomised controlled trials need to be conducted in this area.

Keywords: Pancreatic Enzyme Replacement Therapy; Gastrointestinal agents; pharmacological_interventionpharmacological_intervention;