Table 58Summary of included studies

StudyRegister / Data sourceDatesParticipantsPrevalence: overall results and results by age subgroups - % (n/N)Definition of complication and measurement toolsQuality of the study
Cystic Fibrosis Trust
UK, 2016
Registry annual data report		UK CF Registry20159587 people with cystic fibrosis
  • <16 years: 3845
  • ≥16 years: 5742
Intestinal obstruction:
  • <16 years: 3.0% (116/3845)
  • ≥16 years: 7.4% (423/5742)
  • Overall: 5.6% (539/9587)
Cystic fibrosis care teams at every specialist centre and clinic across the UK filled in forms where they selected what complications (if any) each person with cystic fibrosis had. No definition of complications is provided in the form.Moderate
  • Criteria used to diagnose the complication and consistency of diagnosis across centres were unclear
Data request to UK CF registryUK CF Registry20159587 people with cystic fibrosis
  • Age <16: 3845
  • Age≥16: 5742
Intestinal obstruction in age group <16:
  • Diagnosis of meconium ileus:
  • 6.4% (41/643)
  • No diagnosis of meconium ileus: 2.3% (75/3202)
Intestinal obstruction in age group ≥16:
  • Diagnosis of meconium ileus: 13.9% (113/815)
  • No diagnosis of meconium ileus: 6.3% (310/4927)
Cystic fibrosis care teams at every specialist centre and clinic across the UK entered data on meconium ileus and intestinal obstruction for the registry.Moderate
  • Details from the people in the registry are available in the UK Cystic Fibrosis Trust Registry 2015 Annual Data Report and on the CF Trust website. However criteria used to diagnose the complication and consistency of diagnosis across centres were unclear from these sources
Wiedemann 2001
Germany
Retrospective study		CF Quality Assurance Project registry19973448 people with cystic fibrosis
Age range: 0–58		DIOS
  • Children and young people (age not reported): 3.0%
  • Adults (age not reported): 3.5%
  • All ages: 3.2%
Criteria used to diagnose DIOS not reported.
Centres reported data for each person once a year from a routine visit near the person’s birthday when the person was in a stable clinical condition.		Low
  • Unclear how DIOS was diagnosed and whether it was diagnosed consistently across centres.
  • Age cut-off to define age subgroups was unclear
  • Numerators and denominators not provided.
  • Data was not disaggregated between children and young people

Abbreviations: CF: cystic fibrosis; DIOS: Distal Intestinal Obstruction Syndrome

From: 8, Complications of cystic fibrosis

Cover of Cystic Fibrosis
Cystic Fibrosis: Diagnosis and management.
NICE Guideline, No. 78.
National Guideline Alliance (UK).
Copyright © NICE 2017.

NCBI Bookshelf. A service of the National Library of Medicine, National Institutes of Health.