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Defining DIOS and constipation in cystic fibrosis with a multicentre study on the incidence, characteristics, and treatment of DIOS.
J Pediatr Gastroenterol Nutr 2010; 50(1):38-42JP

Abstract

OBJECTIVES

Various definitions for distal intestinal obstruction syndrome (DIOS), meconium ileus equivalent, and constipation in patients with cystic fibrosis (CF) are used. However, an unequivocal definition for DIOS, meconium ileus equivalent, and constipation is preferred. The aims of this study were, therefore, to seek consensus on the definitions for DIOS and constipation in patients with CF and to determine the incidence, characteristics, and treatment of DIOS in a cohort of paediatric patients with CF.

METHODS

During the 2005 European Society for Paediatric Gastroenterology, Hepatology, and Nutrition meeting in Porto a group of paediatric gastroenterologists discussed the definition of DIOS and constipation in CF. Subsequently, all patients younger than or equal to 18 years with complete DIOS according to the definition agreed upon and diagnosed during the years 2001 to 2005 in 8 CF centres were studied.

RESULTS

Distal intestinal obstruction syndrome was defined as an acute complete or incomplete faecal obstruction in the ileocaecum, whereas constipation was defined as gradual faecal impaction of the total colon. Fifty-one episodes of DIOS in 39 patients were recorded, giving an overall incidence of 6.2 (95% confidence interval, 4.4-7.9) episodes per 1000 patient-years. Of the 39 patients with DIOS, 20% experienced a relapse, 92% were pancreatic insufficient, 44% had a history of meconium ileus at birth, and 82% had a severe genotype. Conservative treatment was effective in 49 of 51 DIOS episodes (96%).

CONCLUSIONS

The European Society for Paediatric Gastroenterology, Hepatology, and Nutrition CF Working Group definitions of DIOS and constipation in CF are specific and make a clear distinction between these 2 entities. The incidence of DIOS in the present study was considerably higher than reported previously.

Authors+Show Affiliations

Wilhelmina Children's Hospital, University Medical Center, Utrecht, the Netherlands.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

19525866

Citation

Houwen, Roderick H., et al. "Defining DIOS and Constipation in Cystic Fibrosis With a Multicentre Study On the Incidence, Characteristics, and Treatment of DIOS." Journal of Pediatric Gastroenterology and Nutrition, vol. 50, no. 1, 2010, pp. 38-42.
Houwen RH, van der Doef HP, Sermet I, et al. Defining DIOS and constipation in cystic fibrosis with a multicentre study on the incidence, characteristics, and treatment of DIOS. J Pediatr Gastroenterol Nutr. 2010;50(1):38-42.
Houwen, R. H., van der Doef, H. P., Sermet, I., Munck, A., Hauser, B., Walkowiak, J., ... Wilschanski, M. (2010). Defining DIOS and constipation in cystic fibrosis with a multicentre study on the incidence, characteristics, and treatment of DIOS. Journal of Pediatric Gastroenterology and Nutrition, 50(1), pp. 38-42. doi:10.1097/MPG.0b013e3181a6e01d.
Houwen RH, et al. Defining DIOS and Constipation in Cystic Fibrosis With a Multicentre Study On the Incidence, Characteristics, and Treatment of DIOS. J Pediatr Gastroenterol Nutr. 2010;50(1):38-42. PubMed PMID: 19525866.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Defining DIOS and constipation in cystic fibrosis with a multicentre study on the incidence, characteristics, and treatment of DIOS. AU - Houwen,Roderick H, AU - van der Doef,Hubert P, AU - Sermet,Isabelle, AU - Munck,Anne, AU - Hauser,Bruno, AU - Walkowiak,Jaroslaw, AU - Robberecht,Eddy, AU - Colombo,Carla, AU - Sinaasappel,Maarten, AU - Wilschanski,Michael, AU - ,, PY - 2009/6/16/entrez PY - 2009/6/16/pubmed PY - 2010/6/18/medline SP - 38 EP - 42 JF - Journal of pediatric gastroenterology and nutrition JO - J. Pediatr. Gastroenterol. Nutr. VL - 50 IS - 1 N2 - OBJECTIVES: Various definitions for distal intestinal obstruction syndrome (DIOS), meconium ileus equivalent, and constipation in patients with cystic fibrosis (CF) are used. However, an unequivocal definition for DIOS, meconium ileus equivalent, and constipation is preferred. The aims of this study were, therefore, to seek consensus on the definitions for DIOS and constipation in patients with CF and to determine the incidence, characteristics, and treatment of DIOS in a cohort of paediatric patients with CF. METHODS: During the 2005 European Society for Paediatric Gastroenterology, Hepatology, and Nutrition meeting in Porto a group of paediatric gastroenterologists discussed the definition of DIOS and constipation in CF. Subsequently, all patients younger than or equal to 18 years with complete DIOS according to the definition agreed upon and diagnosed during the years 2001 to 2005 in 8 CF centres were studied. RESULTS: Distal intestinal obstruction syndrome was defined as an acute complete or incomplete faecal obstruction in the ileocaecum, whereas constipation was defined as gradual faecal impaction of the total colon. Fifty-one episodes of DIOS in 39 patients were recorded, giving an overall incidence of 6.2 (95% confidence interval, 4.4-7.9) episodes per 1000 patient-years. Of the 39 patients with DIOS, 20% experienced a relapse, 92% were pancreatic insufficient, 44% had a history of meconium ileus at birth, and 82% had a severe genotype. Conservative treatment was effective in 49 of 51 DIOS episodes (96%). CONCLUSIONS: The European Society for Paediatric Gastroenterology, Hepatology, and Nutrition CF Working Group definitions of DIOS and constipation in CF are specific and make a clear distinction between these 2 entities. The incidence of DIOS in the present study was considerably higher than reported previously. SN - 1536-4801 UR - https://www.unboundmedicine.com/medline/citation/19525866/Defining_DIOS_and_constipation_in_cystic_fibrosis_with_a_multicentre_study_on_the_incidence_characteristics_and_treatment_of_DIOS_ L2 - http://dx.doi.org/10.1097/MPG.0b013e3181a6e01d DB - PRIME DP - Unbound Medicine ER -