title: Guidelines for the diagnosis and management of distal intestinal obstruction syndrome in cystic fibrosis patients.
abstract: Complete or incomplete intestinal obstruction by viscid faecal material in the terminal ileum and proximal colon - distal intestinal obstruction syndrome (DIOS) - is a common complication in cystic fibrosis. Estimates of prevalence range from 5 to 12 episodes per 1000 patients per year in children, with higher rates reported in adults. DIOS is mainly seen in patients with pancreatic insufficiency, positive history of meconium ileus and previous episodes of DIOS. DIOS is being described with increasing frequency following organ transplantation. Diagnosis is based on suggestive symptoms with a right lower quadrant mass confirmed on X-ray. The main differential is chronic constipation. Treatment consists of rehydration combined with stool softening laxatives or gut lavage with balanced electrolyte solutions. Rapid fluid shifts have been described following osmotic agents. Avoiding dehydration and optimizing pancreatic enzyme dosage may reduce the chance of further episodes. Prophylactic laxative therapy is widely used, but is not evidence-based.
authors.affiliation: Cystic Fibrosis Center, Fondazione IRCCS Cà Granda, Ospedale Maggiore Policlinica, University of Milan, Milan, Italy. carla.colombo@unimi.it
authors.forename: Carla
authors.initials: C
authors.lastname: Colombo
authors.forename: Helmut
authors.initials: H
authors.lastname: Ellemunter
authors.forename: Roderick
authors.initials: R
authors.lastname: Houwen
authors.forename: Anne
authors.initials: A
authors.lastname: Munck
authors.forename: Chris
authors.initials: C
authors.lastname: Taylor
authors.forename: Michael
authors.initials: M
authors.lastname: Wilschanski
mesh_terms: D003106:Colon; D003248:Constipation / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D003550:Cystic Fibrosis / Q000453:epidemiology*; D006801:Humans; D007082:Ileum; D007415:Intestinal Obstruction / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D008511:Medicine / Q000592:standards*; D017410:Practice Guidelines as Topic; D012307:Risk Factors