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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: anne.munck@rdb.aphp.fr.
  • 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.

Abstract

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.

KEYWORDS:

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

PMID:
26927601
DOI:
10.1016/j.jcf.2016.02.002
[PubMed - in process]
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