Defining DIOS and constipation in cystic fibrosis with a multicentre study on the incidence, characteristics, and treatment of DIOS.
OBJECTIVE
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.
Houwen
Roderick H
RH
Wilhelmina Children's Hospital, University Medical Center, Utrecht, the Netherlands.
van der Doef
Hubert P
HP
Sermet
Isabelle
I
Munck
Anne
A
Hauser
Bruno
B
Walkowiak
Jaroslaw
J
Robberecht
Eddy
E
Colombo
Carla
C
Sinaasappel
Maarten
M
Wilschanski
Michael
M
D003248:Constipation / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology; D003550:Cystic Fibrosis / Q000150:complications* / Q000235:genetics; D010188:Exocrine Pancreatic Insufficiency; D005838:Genotype; D006801:Humans; D007077:Ileal Diseases / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology; D045823:Ileus; D015994:Incidence; D007415:Intestinal Obstruction / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology; D008470:Meconium; D015337:Multicenter Studies as Topic