Several recent studies highlight the benefits of multisite collaboration to study infrequent pediatric liver problems. In one of these, it is shown that pancreatic insufficiency (PI) is not a common problem for patients with Alagille syndrome (JPGN 2012; 55: 612-614).
In these studies, the Childhood Liver Disease Research and Education Network (ChiLDREN) collected prospective longitudinal observational date from multiple centers, 16 in this study.
150 subjects who met criteria for Alagille syndrome were enrolled between December 2007 to September 2010. 42 had fecal elastase results available. Elastase results were characterized as normal if >200 μg/g, indeterminant if 100-200 μg/g, and pancreatic insufficient if <100 μg/g.
- 40/42 (95%) had normal results
- 2/40 (5%) were indeterminant
The collaborative study provides a few teaching points:
- Fecal elastase is a very reliable tool for detecting exocrine PI with a 99% negative predictive value for ruling out PI.
- Previous results suggesting that PI was common in Alagille syndrome were flawed due to the fallibility of previous secretin stimulation testing and due to the occurrence of steatorrhea induced by impaired bile salt secretion
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