International prospective study of distal intestinal obstruction syndrome in cystic fibrosis: Associated factors and outcome.
Title | International prospective study of distal intestinal obstruction syndrome in cystic fibrosis: Associated factors and outcome. |
Publication Type | Journal Article |
Year of Publication | 2016 |
Authors | Munck, A., Alberti C., Colombo C., Kashirskaya N., Ellemunter H., Fotoulaki M., Houwen R., Robberecht E., Boizeau P., & Wilschanski M. |
Corporate Authors | CF/Pancreas ESPGHAN Working Group and DIOS Study Group |
Journal | J Cyst Fibros |
Volume | 15 |
Issue | 4 |
Pagination | 531-9 |
Date Published | 2016 07 |
ISSN | 1873-5010 |
Keywords | Adolescent, Adult, Causality, Child, Preschool, Conservative Treatment, Cystic Fibrosis, Digestive System Surgical Procedures, Europe, Female, Hospitalization, Humans, Incidence, Infant, Newborn, Intestinal Obstruction, Longitudinal Studies, Male, Prognosis, Prospective Studies, Risk Factors, Severity of Illness Index |
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. |
DOI | 10.1016/j.jcf.2016.02.002 |
Alternate Journal | J. Cyst. Fibros. |
PubMed ID | 26927601 |