Related Experiment Videos
Meconium ileus equivalent in older patients with cystic fibrosis
Insights
Meconium ileus equivalent, a cystic fibrosis complication, is increasingly seen in adults. Effective medical treatments like acetylcysteine and sodium diatrizoate exist, though prophylactic management remains debated.
Area of Science:
- Pulmonology
- Gastroenterology
- Genetics
Background:
- Cystic fibrosis (CF) survival has improved, leading to increased recognition of adult manifestations.
- Meconium ileus equivalent (MIE) is a less common CF complication, typically affecting older CF patients.
Purpose of the Study:
- To investigate the incidence and characteristics of meconium ileus equivalent in adult CF patients.
- To evaluate the effectiveness of current medical treatments for MIE.
Main Methods:
- Retrospective review of 53 adult patients with cystic fibrosis.
- Analysis of patient records for episodes of meconium ileus equivalent, precipitating factors, and treatment outcomes.
Main Results:
- Eight out of 53 patients experienced 25 episodes of MIE.
- Seven patients had recurrent MIE, with four experiencing at least four episodes.
- All patients responded to medical treatment (acetylcysteine or sodium diatrizoate), including those with prior surgery.
Conclusions:
- Meconium ileus equivalent is a significant issue in adult cystic fibrosis patients.
- Medical management with acetylcysteine or sodium diatrizoate is effective for MIE.
- The role of prophylactic treatment for MIE requires further investigation.
Abstract:
Meconium ileus equivalent is one of the lesser known manifestations of cystic fibrosis, and occurs most often in older patients. With the improved overall survival of patients with cystic fibrosis, one would expect to see this condition more often in the future. Of 53 patients attending our cystic fibrosis clinic for adolescents and adults, eight had experienced a total of 25 episodes of meconium ileus equivalent. Recurrent attacks occurred in seven patients, of whom four had at least four separate, well documented episodes: these episodes were associated with obvious recognised precipitating factors in only three patients. All patients responded to appropriate medical treatment with acetylcysteine or sodium diatrizoate, including three who had previously undergone surgery for meconium ileus equivalent elsewhere. Controversies remain, however, concerning the role of prophylactic medical management with acetylcysteine, sodium diatrizoate, and pancreatic supplements.