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Pancreatic function testing in meconium disease in CF: two case reports
1Department of Pediatrics, Montreal Children's Hospital, McGill University, Canada.
Insights
Cystic fibrosis (CF) meconium disease does not always mean pancreatic insufficiency in infants. Impaired pancreatic fluid secretion, not just enzyme deficiency, may cause intestinal obstruction in CF.
Area of Science:
- Pediatric Gastroenterology
- Cystic Fibrosis Research
- Neonatal Intestinal Disorders
Background:
- Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs, including the pancreas.
- Meconium-related intestinal obstruction is a common early manifestation of CF.
- The role of pancreatic exocrine function in meconium disease is not fully understood.
Observation:
- Two infants with CF presenting with meconium ileus and meconium plug showed no initial signs of pancreatic insufficiency.
- Pancreatic secretory function tests revealed reduced enzyme secretion in both infants.
- One infant with meconium ileus had severely limited pancreatic fluid secretion, while the other with meconium plug had better fluid secretion but poorer proteolytic activity.
Findings:
- Meconium disease in CF infants does not necessitate pancreatic insufficiency or enzyme replacement therapy in infancy.
- Reduced pancreatic enzyme secretion may contribute to meconium disease even without overt pancreatic insufficiency.
- Impaired pancreatic fluid secretion appears to be a significant factor in the pathogenesis of meconium ileus.
Implications:
- Pancreatic exocrine function, particularly fluid secretion, plays a crucial role in preventing meconium-related intestinal obstruction in CF.
- The interplay between intraluminal fluid and pancreatic enzyme activity may determine the risk and severity of intestinal obstruction in CF.
- Further research into pancreatic fluid secretion mechanisms in CF is warranted for improved understanding and management of meconium disease.
Abstract:
We report two infants with cystic fibrosis (CF), presenting with meconium ileus and meconium plug, who had no clinical or biochemical evidence of pancreatic insufficiency during infancy. They underwent pancreatic secretory function testing at 11 and 9 months of age, respectively. Both patients had sufficient lipase and colipase secretion to maintain normal digestion of fat, confirming that meconium disease in CF does not necessarily imply pancreatic insufficiency and the need for enzyme supplementation in infancy. Nonetheless, we documented markedly reduced enzyme secretion in both patients, implying a potential role for the pancreas in the pathogenesis of meconium disease, even when clinical pancreatic insufficiency is absent. In addition, our patient with meconium ileus had a severely limited fluid secretory capacity (10.3% of mean normal values). In contrast, the patient with the milder presentation of meconium plug had a far greater ability to secrete fluid (75% of mean normal), but had poorer pancreatic proteolytic activity. We suggest that impaired fluid secretion may be a very significant factor in the pathogenesis of meconium ileus, and we speculate that an inability to maintain sufficient intraluminal fluid relative to the degree of pancreatic proteolytic deficiency may more adequately explain the risk of occurrence and the severity of intestinal obstruction in CF than either factor alone.