title: International prospective study of distal intestinal obstruction syndrome in cystic fibrosis: Associated factors and outcome.
abstract: 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.
authors.affiliation: Hôpital Robert Debré, Paediatric Gastroenterology and Respiratory Department, CF Centre, University Paris 7, 75019 Paris, France. Electronic address: anne.munck@rdb.aphp.fr.
authors.forename: Anne
authors.initials: A
authors.lastname: Munck
authors.affiliation: Hôpital 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.
authors.forename: Corinne
authors.initials: C
authors.lastname: Alberti
authors.affiliation: Fondazione IRCCS Ca'Granda, Ospedale Maggiore Policlinico, University of Milan, Milan, Italy.
authors.forename: Carla
authors.initials: C
authors.lastname: Colombo
authors.affiliation: Federal State Budgetary Institution "Research Centre for Medical Genetics", Moscow, Russian Federation.
authors.forename: Nataliya
authors.initials: N
authors.lastname: Kashirskaya
authors.affiliation: Medical University, Innsbruck, Austria.
authors.forename: Helmut
authors.initials: H
authors.lastname: Ellemunter
authors.affiliation: University of Thessaloniki, Thessaloniki, Greece.
authors.forename: Maria
authors.initials: M
authors.lastname: Fotoulaki
authors.affiliation: Wilhelmina Children's Hospital, University Medical Centre, Utrecht, the Netherlands.
authors.forename: Roderick
authors.initials: R
authors.lastname: Houwen
authors.affiliation: University Hospital, Ghent, Belgium.
authors.forename: Eddy
authors.initials: E
authors.lastname: Robberecht
authors.affiliation: Hôpital Robert Debré, Clinical Epidemiology Unit, 75019 Paris, France.
authors.forename: Priscilla
authors.initials: P
authors.lastname: Boizeau
authors.affiliation: Hadassah Hebrew University Medical Centre, Jerusalem, Israel.
authors.forename: Michael
authors.initials: M
authors.lastname: Wilschanski
mesh_terms: D000293:Adolescent; D000328:Adult; D015984:Causality; D002675:Child, Preschool; D000072700:Conservative Treatment / Q000379:methods / Q000706:statistics & numerical data; D003550:Cystic Fibrosis / Q000150:complications / Q000453:epidemiology; D013505:Digestive System Surgical Procedures / Q000379:methods / Q000706:statistics & numerical data; D005060:Europe / Q000453:epidemiology; D005260:Female; D006760:Hospitalization / Q000706:statistics & numerical data; D006801:Humans; D015994:Incidence; D007231:Infant, Newborn; D007415:Intestinal Obstruction / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000628:therapy; D008137:Longitudinal Studies; D008297:Male; D011379:Prognosis; D011446:Prospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index
keywords: Abdominal pain; Cystic fibrosis; Distal intestinal obstruction syndrome; Incidence