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Original research
Presentation, characteristics and management of obstructive intestinal conditions in cystic fibrosis
  1. Caitlin Miles1,
  2. Natalie Ling2,
  3. Eldho Paul3,
  4. David Armstrong4,5
  1. 1 Nutrition and Dietetics, Monash Children's Hospital, Melbourne, Victoria, Australia
  2. 2 Department of Medicine, Monash Health, Melbourne, Victoria, Australia
  3. 3 Monash Centre for Health Research and Implementation (MCHRI), Monash University, Melbourne, Victoria, Australia
  4. 4 Department of Respiratory and Sleep Medicine, Monash Children's Hospital, Melbourne, Victoria, Australia
  5. 5 Department of Paediatrics, Monash University, Melbourne, Victoria, Australia
  1. Correspondence to Caitlin Miles, Allied Health, Monash Children's Hospital, Monash Health, Clayton, Victoria, Australia; caitlin.miles{at}


Objective Constipation 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 paediatric and adult CF service over a 12-year period. The secondary aims were to determine the proportion of constipation and DIOS presentations which met the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) CF Working Group definitions and to describe management strategies of both conditions.

Method A retrospective study of children and adults with CF who were admitted with a primary diagnosis of constipation or DIOS between 1 January 2011 and 31 December 2022. ESPGHAN definitions for constipation and DIOS were retrospectively applied to all admissions to determine if the primary medical diagnosis met ESPGHAN criteria.

Results During the 12-year study period, 42 hospitalisations for constipation were recorded in 19 patients, and 33 hospitalisations for DIOS were recorded in 23 patients. 88.10% of constipation episodes met ESPGHAN definitions, compared with 3.0% of DIOS episodes. Constipation and DIOS were primarily treated with polyethylene glycol (PEG). The use of sodium amidotrizoate meglumine enemas was significantly higher in the DIOS group (p=0.045). Those admitted with DIOS were significantly less likely to be recommended a weaning dose of PEG (p=0.018).

Conclusion Children and adults with CF are more commonly admitted for the management of constipation than DIOS. There is considerable variation in diagnostic and therapeutic practice, and this study highlights the need to enhance the translation and adoption of existing best-practice guidelines.

  • cystic fibrosis
  • constipation
  • gastrointestinal tract
  • small bowel
  • colonic diseases

Data availability statement

All data relevant to the study are included in the article or uploaded as supplementary information.

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Data availability statement

All data relevant to the study are included in the article or uploaded as supplementary information.

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  • Contributors CM: content guarantor, conceptualisation, methodology and design, investigation and acquisition of research data, research output and writing—original manuscript. NL: investigation and acquisition of research data. EP: formal analysis of research data DA: conceptualisation, methodology and design, research output and writing—review and editing of original manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.