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J Cyst Fibros. 2016 Jul;15(4):531-9. doi: 10.1016/j.jcf.2016.02.002. Epub 2016 Feb 23.

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

Author information

  • 1Hôpital Robert Debré, Paediatric Gastroenterology and Respiratory Department, CF Centre, University Paris 7, 75019 Paris, France. Electronic address:
  • 2Hôpital Robert Debré, Clinical Epidemiology Unit, 75019 Paris, France; Sorbonne Paris Cité, UMR-S1123, ECEVE, University Paris Diderot, 75019 Paris, France; Inserm U1123 and CIC-EC 1426, 75019 Paris, France.
  • 3Fondazione IRCCS Ca'Granda, Ospedale Maggiore Policlinico, University of Milan, Milan, Italy.
  • 4Federal State Budgetary Institution "Research Centre for Medical Genetics", Moscow, Russian Federation.
  • 5Medical University, Innsbruck, Austria.
  • 6University of Thessaloniki, Thessaloniki, Greece.
  • 7Wilhelmina Children's Hospital, University Medical Centre, Utrecht, the Netherlands.
  • 8University Hospital, Ghent, Belgium.
  • 9Hôpital Robert Debré, Clinical Epidemiology Unit, 75019 Paris, France.
  • 10Hadassah Hebrew University Medical Centre, Jerusalem, Israel.



Distal intestinal obstruction syndrome (DIOS) is a specific complication of cystic fibrosis.


A study was performed in 10 countries to prospectively evaluate the incidence, associated factors, and treatment modalities in children and adults.


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).


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.


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

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