Academic Journal

Defining DIOS and constipation in cystic fibrosis with a multicentre study on the incidence, characteristics, and treatment of DIOS

التفاصيل البيبلوغرافية
العنوان: Defining DIOS and constipation in cystic fibrosis with a multicentre study on the incidence, characteristics, and treatment of DIOS
المؤلفون: R. H. J. Houwen, H. P. J. Van Der Doef, I. Sermet, A. Munck, B. Hauser, J. Walkowiak, E. Robberecht, M. Sinaasappel, M. Wilschanski, ESPGHAN Cystic Fibrosis Working Group, C. Colombo
المساهمون: R.H.J. Houwen, H.P.J. Van Der Doef, I. Sermet, A. Munck, B. Hauser, J. Walkowiak, E. Robberecht, C. Colombo, M. Sinaasappel, M. Wilschanski, ESPGHAN Cystic Fibrosis Working Group
بيانات النشر: Lippincott Williams & Wilkins
سنة النشر: 2010
المجموعة: The University of Milan: Archivio Istituzionale della Ricerca (AIR)
مصطلحات موضوعية: Settore MED/38 - Pediatria Generale e Specialistica, Settore MED/12 - Gastroenterologia
الوصف: 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. Copyright
نوع الوثيقة: article in journal/newspaper
اللغة: English
Relation: info:eu-repo/semantics/altIdentifier/pmid/19525866; info:eu-repo/semantics/altIdentifier/wos/WOS:000273173700013; volume:50; issue:1; firstpage:38; lastpage:42; journal:JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION; http://hdl.handle.net/2434/168495; info:eu-repo/semantics/altIdentifier/scopus/2-s2.0-76149106847
DOI: 10.1097/MPG.0b013e3181a6e01d
الاتاحة: http://hdl.handle.net/2434/168495
https://doi.org/10.1097/MPG.0b013e3181a6e01d
رقم الانضمام: edsbas.32BB4C8C
قاعدة البيانات: BASE
الوصف
DOI:10.1097/MPG.0b013e3181a6e01d