Distal Intestinal Obstruction Syndrome Research Articles

Cystic fibrosis and fat malabsorption: Pathophysiology of the cystic fibrosis gastrointestinal tract and the impact of highly effective CFTR modulator therapy.

Cystic fibrosis (CF) is a progressive, genetic, multi-organ disease affecting the respiratory, digestive, endocrine, and reproductive systems. CF can affect any aspect of the gastrointestinal (GI) tract, including the esophagus, stomach, small intestine, colon, pancreas, liver, and gall bladder. GI pathophysiology associated with CF results from CF membrane conductance regulator (CFTR) dysfunction. The majority of people with CF (pwCF) experience exocrine pancreatic insufficiency resulting in malabsorption of nutrients and malnutrition. Additionally, other factors can cause or worsen fat malabsorption, including the potential for short gut syndrome with a history of meconium ileus, hepatobiliary diseases, and disrupted intraluminal factors, such as inadequate bile salts, abnormal pH, intestinal microbiome changes, and small intestinal bacterial overgrowth. Signs and symptoms associated with fat malabsorption, such as abdominal pain, bloating, malodorous flatus, gastroesophageal reflux, nausea, anorexia, steatorrhea, constipation, and distal intestinal obstruction syndrome, are seen in pwCF despite the use of pancreatic enzyme replacement therapy. Given the association of poor nutrition status with lung function decline and increased mortality, aggressive nutrition support is essential in CF care to optimize growth in children and to achieve and maintain a healthy body mass index in adults. The introduction of highly effective CFTR modulator therapy and other advances in CF care have profoundly changed the course of CF management. However, GI symptoms in some pwCF may persist. The use of current knowledge of the pathophysiology of the CF GI tract as well as appropriate, individualized management of GI symptoms continue to be integral components of care for pwCF.

Specifics of cystic fibrosis genetic spectrum in Georgia

Cystic fibrosis (CF) is a life-threatening autosomal recessive disease caused by mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR). F508del is the most common mutation in the world. Other mutations are rare and population specific. The study aimed to comprehensively analyze the distribution of CFTR mutations in Georgian CF patients. Knowing the prevalence and characteristics of specific mutations can be reflected in genetic counseling and management strategies. We reviewed the data of 129 CF patients, aged < 18 years, from all parts of Georgia. 91 patients with a clinically confirmed CF diagnosis were tested for CF-causing mutations (constituting 70.5% of all currently registered CF patients in the country). These patients have been analyzed for rare CFTR variants by massively parallel sequencing of the entire CFTR coding region and adjacent introns combined with the analysis of intra-CFTR rearrangements. CFTR gene analysis revealed 29 mutations in Georgian CF patients. The most common mutation was c.1545_1546delTA (1677delTA) with a frequency of 42.7%, while the second most common mutation, W1282X, was detected in 11.2% of all CF alleles. Another 27 CFTR mutations have low frequency, including F508del (6.7% of alleles). 3 novel mutations were found (c.708dupT; CFTRdele16_17; c.3170C>G) and reported to CFTR2 database.According to the data, the distribution of CFTR mutations in the Georgian CF population differs regarding the high frequency of mutation c.1545_1546delTA (1677delTA) and the low frequency of the predominant F508del mutation. Compared to patients with F508 del and W1282X mutations, patients with 1677delTA have typical manifestations and complications; however, the frequency of growth retardation and liver damage is 3 times, and the frequency of chronic respiratory manifestations and chronic malnutrition is 2 times lower, though pancreatic insufficiency is more severe in patients with 1677delTa. CF-associated diabetes, distal intestinal obstruction syndrome, and hemoptysis were only observed in patients with the 1677delTA mutation. The mortality rate is lower compared to patients with F508del and W1282X mutations.

Presentation, characteristics and management of obstructive intestinal conditions in cystic fibrosis

ObjectiveConstipation and distal intestinal obstruction syndrome (DIOS) are common gastrointestinal manifestations of cystic fibrosis (CF). The primary aim was to describe the characteristics of constipation and DIOS hospitalisations in a...

Palliative care needs among outpatient adults with cystic fibrosis: Baseline data from the Improving Life with CF trial

BackgroundLittle is known about the burden of illness experienced by people with cystic fibrosis (pwCF) since the advent of CF transmembrane conductance regulator (CFTR) modulator therapies. Studies that characterize the nature of illness burden are needed to inform the development and implementation of palliative care programs that can serve this population and address quality of life concerns. MethodsAdults with CF treated at five U.S. CF centers were surveyed to obtain baseline data for the Improving Life with CF primary palliative care implementation trial. Consenting patients completed the Integrated Palliative Care Outcome Scale (IPOS), a multidimensional measure of unmet needs for palliative care. Sociodemographic and clinical information was also obtained. The associations among these variables were examined through bivariate and multivariable analyses. ResultsAmong 256 adults, the most distressing symptoms included not feeling “at peace”, communication difficulties with family/friends, anxiety over illness or its treatment, and a lack of energy. In the multivariable analyses, CFTR modulator use was associated with lower IPOS total and physical symptoms scores; female sex and increased hospitalizations were associated with higher scores. Increased age and history of distal intestinal obstructive syndrome were associated with higher IPOS physical symptoms scores. ConclusionsThese findings illuminate the nature of illness burden for pwCF in the era of CFTR modulator therapies. Although illness burden is positively affected by modulator therapy, there is a continuing need for palliative care to address physical, emotional, and spiritual distress, and the communication and practical needs experienced by adults with CF.

Distal Intestinal Obstruction Syndrome in Patients with Cystic Fibrosis: Two Separate Cases in the Pediatric Intensive Care Unit

Distal Intestinal Obstruction Syndrome in Patients with Cystic Fibrosis: Two Separate Cases in the Pediatric Intensive Care Unit

P289 Intestinal obstruction syndromes in cystic fibrosis

Meconium ileus (MI) presents at birth as small bowel obstruction. Distal Intestinal Obstruction Syndrome (DIOS) is a faecal obstruction of the ileocaecum after the neonatal period. We undertook a retrospective review of all patients at our CF Centre to characterise the spectrum of intestinal syndromes.

Effectiveness of an Oral Gastrografin Regimen for Complete Distal Intestinal Obstruction Syndrome.

To assess the effectiveness of oral Gastrografin treatment and outcomes in adult patients with complete distal intestinal obstruction syndrome (cDIOS). DIOS is an important gastrointestinal complication of cystic fibrosis (CF). Conservative treatment options for cDIOS are largely empirical, and the optimal management remains unclear. Surgery should be reserved for patients who have failed nonoperative treatment or have immediate indications for surgery. A retrospective single-institution cohort study was undertaken of adults with CF who had undergone lung transplantation and were admitted with an episode of cDIOS between 2004 and 2020. The outcomes of treatment in a high-volume CF transplant center with routine oral Gastrografin-based therapy were assessed. Forty-seven episodes of cDIOS were recorded in 29 (23.3%) of 124 patients who had undergone lung transplantation for CF, and mean age at cDIOS was 30.3 years (SD ±11.2). Mean follow-up post cDIOS was 75.6 months (SD ±45.5). Twelve patients had >1 cDIOS episode. One episode occurred during recovery after transplantation, and 5 patients were readmitted within 30 days posttransplant with cDIOS. A history of previous abdominal surgery was associated with the development of cDIOS (P < 0.001). Oral Gastrografin therapy was used in 95.7% of the episodes, at varying doses. Three patients (7.0%) were resistant to oral Gastrografin treatment, requiring laparotomy. There were no deaths due to DIOS. Oral Gastrografin is effective and safe for the treatment of cDIOS, with low treatment failure rates. It should be considered as a first-line treatment option for patients with CF presenting with complete distal intestinal obstruction.

Applying patient personalized organoid model system to understand complex GI manifestations in cystic fibrosis disease

Background: Stem cell-derived organotypic culture has become a revolutionary tool in transforming care for patients with multiple GI-disorders. Cystic fibrosis is a deadly genetic disorder with multiple GI complications, such as Distal intestinal obstruction syndrome (DIOS), chronic constipation, Gastroesophageal reflux disease (GERD), and in some cases pancreatitis, caused by loss of function mutations in the gene that encodes for the cystic fibrosis transmembrane conductance regulator (CFTR) chloride/bicarbonate ion channel. Due to genetic and environmental influences, CF patients exhibit large phenotypic variation. These factors make clinical trials difficult and largely impractical due to limited and heterogenous patient pools, and additionally, the benefit of approved small-molecule CF therapy in a large number of rare mutation patients is not known. Moreover, critical questions of whether CFTR contributes to the development of intestine and how it contributes to GI-related issues remain unanswered. Results: In this study, we performed a comprehensive bench-side study using in vitro patient enteroids and in vivo mice implanted Human Intestinal Organoids (HIOs) to test highly effective CFTR modulators -Ivacaftor response for a rare CF mutation patient. Based on the positive Ivacaftor response in the enteroids, the patient was enrolled in N=1 clinical trial and showed improved GI outcomes upon Ivacaftor treatment. HIO implantation model allowed in vivo modulator dosing and provided an elegant human organ-based demonstration of bench-to-bedside testing of modulator effects. Additionally, using the CF HIO model the role of CFTR function in the maturation of human intestine was reported for the first time. In HIOs derived from a stop mutation CF patient and CFTR-corrected HIOs as control, we performed single cell RNAseq analysis to generate a molecular map of GI-cell type specific transcriptomic alterations and identify key targetable pathways relevant to stop mutations in CFTR for which currently there is no small molecule therapy. Conclusion: In all, we demonstrate that these models effectively serve to translate data from the lab to the clinic and back so that patient specific therapies could be validated, or new therapies be devised. Additionally, single-cell RNA sequencing in patient HIOs will provide useful insights into cell population specific and transcriptomic level alterations that are associated with abnormalities in GI function in CF. Nothing to disclose This is the full abstract presented at the American Physiology Summit 2023 meeting and is only available in HTML format. There are no additional versions or additional content available for this abstract. Physiology was not involved in the peer review process.

Distal intestinal obstruction syndrome in a child with cystic fibrosis

The aim of the study – to analyze the clinical case of a child with distal intestinal obstruction syndrome in cystic fibrosis. A 9.5 year old patient was admitted to the Gastroenterology Department of the Republican Specialized Scientific and Practical Center of Pediatrics with complaints of an increase in abdominal circumference, increased peristalsis, weakness, lethargy, swelling in the limbs, loose stools, and weight loss. From the anamnesis: from the age of 6 years it was observed periodic vomiting, not associated with food intake. At the age of 8 years, the condition worsened, bloating joined the vomiting. At the place of residence, the diagnosis was made “Acute intestinal obstruction. Volvulus of the sigmoid colon”. Over the next 6 months, the condition was relatively satisfactory, and then stool disorders began – 3–4 times a day and bloating. Considering the presence of steatorrhea, changes in the pancreas on ultrasound and MSCT, the child was referred to a geneticist, the result of a sweat test was 86.0 mEq/l. The diagnosis was made: Cystic fibrosis, mixed form. Syndrome of distal intestinal obstruction. Partial intestinal obstruction. Severe PEI, celiac disease and HIV were excluded. On the background of the therapy, the child’s condition improved relatively. Within a month after discharge, the condition was satisfactory, and then worsened again. After 3 months, the girl was re-hospitalized with the same complaints. After consulting the surgeons, recommendations were given to perform the operation due to the appearance of signs of partial lower intestinal obstruction, but the relatives signed a voluntary refusal of the operation, the child was taken home against receipt. The girl was discharged with the final diagnosis: Cystic fibrosis, mixed form, severe course. Syndrome of distal intestinal obstruction. Partial lower intestinal obstruction. Syndrome of exudative enteropathy, severe PEI. The girl died 2 weeks after discharge. Distal intestinal obstruction syndrome (DIOS) may be misunderstood by those who are not familiar with cystic fibrosis. Pediatric gastroenterologists, surgeons should be alert when this condition is detected in combination with other symptoms indicating cystic fibrosis.

Intussusception of the appendix in a young adult: an important differential diagnosis of abdominal pain in cystic fibrosis patients?

Cystic fibrosis (CF) is commonly associated with gastrointestinal manifestations from infancy to adulthood. Distal intestinal obstruction syndrome (DIOS) affects 20% of CF patients, where intussusception can be a rare complication. A 20-year-old CF male was diagnosed with a 3-day history of right iliac fossa pain and diarrhoea. Clinical examination revealed a tender palpable mass in the right iliac fossa and raised serum inflammatory markers. Contrast computerized-tomography of the abdomen-pelvis suggested intussusception of the appendix and further confirmed on histological analyses. The patient underwent an open appendicectomy where the intussusception had self-resolved. The literature review indicated a scarcity of data with 10 cases reported of intussusception in adult CF patients. Our case was in line with previous research of transient intussusception. This rare case highlights an importance to carry a higher index of suspicion for gastrointestinal manifestations in CF patients where differential diagnoses of DIOS and intussusception should be considered in the acute presentation.

Selective isoxazolopyrimidine PAT1 (SLC26A6) inhibitors for therapy of intestinal disorders

This study reports identification of selective isoxazolopyrimidine inhibitors of intestinal anion exchanger SLC26A6 (PAT1) with improved potency and good in vivo efficacy. PAT1 inhibitors are novel drug candidates for intestinal diseases.

S363 Successful Treatment of Distal Intestinal Obstruction Syndrome With N-acetylcysteine and Polyethylene Glycol via Colonoscopy

Introduction: Cystic fibrosis (CF) can lead to obstructive gastrointestinal disease, such as meconium ileus in children and distal intestinal obstruction syndrome (DIOS) in adults. DIOS is characterized by the accumulation of inspissated stool and viscous secretions in the terminal ileum and proximal colon, leading to complete or incomplete obstruction. Treatment strategies for DIOS are still essentially empirical as there are no randomized controlled trials to guide therapy. We describe a case of a CF patient with DIOS successfully treated with 4% N-acetylcysteine (NAC) and polyethylene glycol (PEG) administered via colonoscopy. Case Description/Methods: A 46-year-old woman with cystic fibrosis and prior episodes of DIOS presented with several days of abdominal pain and constipation. On exam, her abdomen was distended and diffusely tender to palpation. Abdominal CT showed extensive debris in the distal small bowel with resultant dilation proximally, thickening at the terminal ileum, and a decompressed colon (Fig A). She was initially managed with frequent enemas and oral laxatives including Lubiprostone and PEG via nasogastric tube. Her symptoms worsened and she was taken for urgent colonoscopy. With the patient in supine position, the colonoscope was meticulously advanced to the cecum, traversing several matted, mucoid-adherent folds (Fig B). A mucoid plug was seen extending from the ileocecal (Fig B) valve to the walls of the cecum (Fig C). The mucoid plug was injected with 4% NAC and PEG. The scope was then advanced into the terminal ileum approximately 20 cm to the proximal end of a large, actively dissolving fecalith with administration of NAC and PEG. A CRE 8-9-10 mm balloon was used to sweep the stool from the distal ileum. Following this maneuver, peristalsis was observed with inspissated stool emanating from the distal ileum towards the IC valve. Discussion: Optimal management of DIOS in CF patients remains a challenge. Although most cases are relieved with conservative treatments, select patients will need surgery, which is associated with a higher peri- and postoperative morbidity. However, the administration of 4% N-acetylcysteine and polyethylene glycol via colonoscopy offers a viable remedy that may obviate the need for surgery. Watch the video at https://tinyurl.com/ACGAbstractS363AFigure 1.: (A) Abdominal CT showing extensive debris in the distal small bowel. (B) Matted, mucoid-adherent folds in the colon. (C) A mucoid plug extending from the ileocecal valve to the walls of the cecum.

1052 Intussusception of the Appendix in a Young Adult with Cystic Fibrosis: An Important Differential Diagnosis of Abdominal Pain in Cystic Fibrosis Patients?

Abstract Background Cystic fibrosis (CF) is commonly associated with gastrointestinal manifestations from infancy to adulthood. Intussusception in the paediatric CF population is widely reported, whereas in the adult, distal intestinal obstruction syndrome (DIOS) is common affecting 20%, of which, intussusception is rare and appendiceal intussusception is rarer, affecting 1%. Case-Description A 20-year-old male with CF presented with 3 days of right iliac fossa pain and diarrhoea. On admission, he was hypotensive and afebrile. Clinical examination revealed tenderness of the right flank with a palpable mass in the right iliac fossa. Serum investigations showed mildly raised inflammatory markers. Contrast CT of the abdomen-pelvis confirmed intussusception of the appendix into the ascending colon. Histological analysis of the appendix further demonstrated intussusception, with intraluminal mucinous material. Fascinatingly, no transmural inflammation was present. The patient underwent a laparotomy-open appendicectomy. Interestingly, intraoperative findings showed the intussusception resolved, no longer requiring surgical reduction. Patient postoperative recovery was complicated by pneumonia for which he was transferred for specialist CF respiratory care and thereafter, discharged. Discussion Literature review highlighted a paucity of data, with 10 reported cases of appendiceal intussusception in adult CF patients. Interestingly, we report the intussusception had reduced by the time of operation. This is in-keeping with previous case reports of transient intussusception which spontaneously resolved. Conclusion From this rare case presentation, we have learnt it is imperative to carry a high index of suspicion for gastrointestinal manifestations in CF patients where acute appendicitis is seen less often and differential diagnoses like DIOS and intussusception should be considered.

Cystic Fibrosis Modulator Therapies.

Cystic fibrosis (CF) is an inherited multisystemic disease that can cause progressive bronchiectasis, pancreatic endocrine and exocrine insufficiency, distal intestinal obstruction syndrome, liver dysfunction, and other disorders. Traditional therapies focused on the treatment or prevention of damage to each organ system with incremental modalities such as nebulized medications for the lungs, insulin for diabetes, and supplementation with pancreatic enzymes. However, the advent of highly effective modulator therapies that target specific cystic fibrosis transmembrane conductance regulator protein malformations resulting from individual genetic mutations has transformed the lives and prognosis for persons with CF.

Surgical considerations in cystic fibrosis: what every general surgeon needs to know.

Cystic fibrosis (CF) is a complex multiorgan disease, which often affects the gastrointestinal tract. With improved CF specific therapies and multidisciplinary management, patients with CF are now living longer with a median life expectancy of around 50 years. This increased life expectancy has resulted in corresponding increase in presentations of the CF patient with comorbid surgical conditions that were never important considerations. Investigations and management of these conditions, such as distal intestinal obstruction syndrome and colorectal cancer warrant good clinical understanding of the unique challenges that CF patients present including chronic immunosuppression, impaired respiratory function and their multi-organ dysfunction. The purpose of this review is to provide general surgeons with a contemporary update on the CF related surgical issues as they are likely to become increasingly involved in the care of these complex patients and form an integral part of the multidisciplinary team.

P171 Improved diagnosis and treatment of distal intestinal obstruction syndrome (DIOS) with a simplified treatment routine

P171 Improved diagnosis and treatment of distal intestinal obstruction syndrome (DIOS) with a simplified treatment routine

Two systematic cochrane reviews of the prevention and treatment of distal intestinal obstruction syndrome in cystic fibrosis

Two systematic cochrane reviews of the prevention and treatment of distal intestinal obstruction syndrome in cystic fibrosis

Distal intestinal obstruction syndrome as a presenting manifestation of cystic fibrosis in an infant

Distal intestinal obstruction syndrome as a presenting manifestation of cystic fibrosis in an infant

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

Cystic fibrosis (CF) is the most common, life-limiting, genetically inherited disease. It affects multiple organs, particularly the respiratory system. However, gastrointestinal problems such as constipation and distal intestinal obstruction syndrome (DIOS) are also important and well-recognised complications in CF. They share similar symptoms e.g. bloating, abdominal pain, but are distinct conditions. Constipation occurs when there is gradual faecal impaction of the colon, but DIOS occurs when there is an accumulation of faeces and sticky mucus, forming a mass in the distal part of the small intestine. The mass may partially block the intestine (incomplete DIOS) or completely block the intestine (complete DIOS). Symptoms of DIOS can affect quality of life and other aspects of CF health, such as airway clearance, exercise, sleep and nutritional status. Treatment of constipation and prevention of complete bowel obstruction are required for gastrointestinal management in CF. However, many different strategies are used in clinical practice and there is a lack of consensus. The importance of this topic was highlighted in a recent research priority setting exercise by the James Lind Alliance. To evaluate the effectiveness and safety of laxative agents of differing types for preventing DIOS (complete and incomplete) in children and adults with CF. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. Date of search: 09 September 2021. We also searched online trial registries. Date of last search: 12 October 2021. Randomised and quasi-randomised controlled parallel trials comparing laxative therapy for preventing DIOS (including osmotic agents, stimulants, mucolytics and substances with more than one action) at any dose to placebo, no treatment or an alternative laxative therapy, in people of any age with pancreatic sufficient or insufficient CF and any stage of lung disease. Randomised cross-over trials were judged on an individual basis. Two authors independently assessed trials for inclusion, extracted outcome data and performed a risk of bias assessment for the included data. We judged the certaintyof the evidence using GRADE criteria. We included one cross-over trial (17 participants) with a duration of 12 months, in which participants were randomly allocated to either cisapride (a gastro-prokinetic agent) or placebo for six months each. The trial had an unclear risk of bias for most domains but had a high risk of reporting bias. Radiograph scores revealed no difference in occurrence of DIOS between cisapride and placebo (narrative report, no data provided). There were no adverse effects. Symptom scores were the only secondary outcome within the review that were reported. Total gastrointestinal symptom scores favoured cisapride with a statistically significant mean difference (MD) of -7.60 (95% confidence interval (CI) -14.73 to -0.47). There was no significant difference at six months between cisapride and placebo for abdominal distension, MD -0.90 (95% CI -2.39 to 0.59) or abdominal pain, MD -0.4 (95% CI -2.05 to 1.25). The global symptom scores (whether individuals felt better or worse) were reported in the paper to favour cisapride and be statistically significant (P < 0.05). We assessed the available data to be very low certainty. There was a great deal of missing data from the included trial and the investigators failed to report numerical data for many outcomes. The overall risk of bias of the trial was unclear and it had a high risk for reporting bias. There was also indirectness; the trial drug (cisapride) has since been removed from the market in several countries due to adverse effects, thus it has no current applicability for preventing DIOS. The included trial also had very few participants, which downgraded the certaintya further level for precision. There is an absence of evidence for interventions for the prevention of DIOS. As there was only one included trial, we could not perform a meta-analysis of the data. Furthermore, the included trial compared a prokinetic agent (cisapride) that is no longer licensed for use in a number of countries due to the risk of serious cardiac events, a finding that came to light after the trial was conducted. Therefore, the limited findings from the trial are not applicable in current clinical practice. Overall, a great deal more research needs to be undertaken on gastrointestinal complications in CF, as this is a very poorly studied area compared to respiratory complications in CF.

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

Cystic fibrosis is the most common life-limiting autosomal recessive genetic disorder in white populations. Distal intestinal obstruction syndrome (DIOS) is an important morbidity in cystic fibrosis. It is the result of the accumulation of viscid faecal material within the bowel which combines with thick, sticky mucus produced in the intestines of people with cystic fibrosis. The intestine may be completely blocked (complete DIOS) or only partially blocked (incomplete DIOS). Once a diagnosis of DIOS has been made, the goal of therapy is to relieve the acute complete or incomplete faecal obstruction and ultimately prevent the need for surgical intervention. This review aimed to evaluate the effectiveness and safety of different treatment regimens for the treatment of DIOS (complete and incomplete) in children and adults with cystic fibrosis. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. Date of search: 09 September 2021. We also searched online trial registries. Date of last search: 12 October 2021. Randomised controlled trials, quasi-randomised controlled trials (including cross-over trials (to be judged on an individual basis)) comparing the use of laxative agents or surgery for treating DIOS in children, young people and adults with cystic fibrosis to each other, placebo or no intervention. Two authors independently screened papers, extracted trial details and assessed for risk of bias. The authors assessed the quality of evidence using GRADE. 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. 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.