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Abstract:
Meconium ileus equivalent (MIE) can be defined as a clinical manifestation in cystic fibrosis (CF) patients caused by acute intestinal obstruction by putty-like faecal material in the cecum or terminal ileum. A broader definition includes a more chronic condition in CF patients with abdominal pain and a coecal mass which may eventually pass spontaneously. The condition occurs only in CF patients with exocrine pancreatic insufficiency (EPI). It has not been seen in other CF patients nor in non-CF patients with EPI. The frequency of these symptoms has been reported as 2.4%-25%. Pathophysiologically, MIE is probably caused by a combination of EPI, increased intestinal transit time, and abnormal intestinal mucus. The treatment should primarily be non-operative. Specific treatment with N-acetylcysteine, administrated orally and/or as an enema is recommended. Enemas with the water soluble contrast medium, meglucamine diatrizoate (Gastrografin), provide an alternative form for treatment and can also serve diagnostic purposes. It is important that the physician is familiar with this disease entity and the appropriate treatment with the above mentioned drugs. Non-operative treatment is often effective, and dangerous complications following surgery can thus be avoided.
Insights
Meconium ileus equivalent (MIE) is a cystic fibrosis (CF) complication causing intestinal obstruction. Non-operative treatments like N-acetylcysteine or Gastrografin enemas are effective and recommended for CF patients with exocrine pancreatic insufficiency.
Area of Science:
- Gastroenterology
- Pediatrics
- Genetics
Background:
- Meconium ileus equivalent (MIE) is a specific intestinal obstruction in cystic fibrosis (CF) patients.
- It exclusively affects CF patients with exocrine pancreatic insufficiency (EPI).
Observation:
- MIE presents as acute or chronic intestinal obstruction due to impacted fecal material.
- Symptoms include abdominal pain and a palpable cecal mass, occurring in 2.4%-25% of CF patients.
Findings:
- Pathophysiology involves EPI, prolonged intestinal transit, and abnormal mucus.
- Non-operative management is the primary treatment strategy.
Implications:
- N-acetylcysteine (oral or enema) and meglumine diatrizoate (Gastrografin) enemas are recommended treatments.
- Physician familiarity with MIE and its non-operative management can prevent surgical complications.