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Meconium ileus equivalent in children with cystic fibrosis
Insights
Children with cystic fibrosis can experience intestinal obstruction later in life due to thickened bowel contents. Increased survival rates mean more patients may face this complication in adolescence and adulthood.
Area of Science:
- Pediatric Gastroenterology
- Pulmonology
- Genetics
Background:
- Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs.
- Intestinal obstruction is a known complication, often presenting in infancy as meconium ileus.
- Improved CF management increases long-term survival, altering disease presentation patterns.
Observation:
- This report details two cases of intestinal obstruction in children with CF at ages 5 and 14.
- Obstruction was attributed to inspissated (thickened) small bowel content.
- Precipitating factors included reduced pancreatic enzyme supplementation, dehydration, and chest infections.
Findings:
- Late-onset intestinal obstruction, termed meconium ileus equivalent, can occur in CF patients beyond infancy.
- This complication is linked to factors affecting intestinal fluid and enzyme balance.
- The incidence of late-onset obstruction may rise with improved CF survival.
Implications:
- Increased awareness of meconium ileus equivalent is crucial for pediatricians and gastroenterologists managing CF.
- Early recognition and intervention can prevent severe complications.
- Longitudinal care strategies for CF should address the risk of late-onset intestinal issues.
Abstract:
Children with cystic fibrosis may develop intestinal obstruction later in life due to inspissation of small bowel content. Two children with cystic fibrosis who developed intestinal obstruction at the age of 5 and 14 years respectively are reported. The condition may be precipitated by reduction or stopping pancreatic supplement, dehydration or intercurrent chest infection. The intensive therapy of chest complications has improved the survival of children with cystic fibrosis, so that it is likely that in future more cases of meconium ileus equivalent will present in later childhood and in adulthood.