International prospective study of distal intestinal obstruction syndrome in cystic fibrosis: Associated factors and outcome

Anne Munck, Corinne Alberti, Carla Colombo, Nataliya Kashirskaya, Helmut Ellemunter, Maria Fotoulaki, Roderick Houwen, Eddy Robberecht, Priscilla Boizeau, Michael Wilschanski

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Background Distal intestinal obstruction syndrome (DIOS) is a specific complication of cystic fibrosis. Methods A study was performed in 10 countries to prospectively evaluate the incidence, associated factors, and treatment modalities in children and adults. Results 102 patients presented 112 episodes. The incidence of DIOS was similar in children and adults. Medical treatment failed only in cases of complete DIOS (11%). Children with meconium ileus had a higher rate of surgery for DIOS (15% vs. 2%, p = 0.02). Complete DIOS entailed longer hospitalisation (4 [3; 7] days vs. 3 [1; 4], p = 0.002). Delayed arrival at hospital and prior weight loss had a significant impact on the time needed for DIOS resolution. Associated CF co-morbidities for DIOS included meconium ileus (40% vs. 18%, p < 0.0001), exocrine pancreatic insufficiency (92% vs. 84%, p = 0.03), liver disease (22% vs. 12%, p = 0.004), diabetes mellitus (49% vs. 25%, p = 0.0003), and Pseudomonas aeruginosa (68% vs. 52%, p = 0.01); low fibre intake and insufficient hydration were frequently observed. Female gender was associated with recurrent DIOS (75% vs. 52%, p = 0.04), constipation with incomplete episodes (39% vs. 11%, p = 0.03), and poor patient compliance in taking pancreatic enzyme therapy during complete episodes (25% vs. 3%, p = 0.02). Conclusion DIOS is a multifactorial condition having a similar incidence in children and adults. We show that delayed arrival at hospital after the initial symptoms causes significant morbidity. Early recognition and treatment would improve the prognosis.
Original languageEnglish
Pages (from-to)531-539
JournalJournal of Cystic Fibrosis
Issue number4
Publication statusPublished - Jul 2016


  • Cystic fibrosis
  • Abdominal pain
  • Distal intestinal obstruction syndrome
  • Incidence


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