Diagnostic Performance of Fecal Elastase and Risk Factors for Exocrine Pancreatic Insufficiency in Children with Pancreatitis: An INSPPIRE-2 Study.

OBJECTIVE: To evaluate the diagnostic performance of fecal elastase (FE) in exocrine pancreatic insufficiency (EPI) and examine the risk factors for EPI in children with acute recurrent pancreatitis (ARP) or chronic pancreatitis (CP). STUDY DESIGN: We analyzed prospectively collected demographic, clinical, and EPI data of children with ARP or CP (n=1007) enrolled in the INSPPIRE-2 (INternational Study group of Pediatric Pancreatitis: In search for a cuRE) consortium. FE performance was assessed against individual markers of fat malabsorption and a composite reference standard in which the presence of any 1 of the following was considered consistent with fat malabsorption in lieu of a gold-standard pancreas function test: (1) clinical diagnosis of EPI, (2) vitamin A or E deficiency, or (3) BMI z-score ≤-2. Cox regression models were used to identify predictors of EPI. RESULTS: EPI was diagnosed in 195/1007 (19.4%) children with ARP/CP, with FE being the most commonly used diagnostic tool. FE demonstrated low sensitivity (55.8% and 65.1%), moderate specificity (82.8% and 75.5%), and a high negative predictive value (92% and 93%), at cut-offs of 100 μg/g and 200 μg/g stool, respectively, in detecting at least 1 marker of fat malabsorption. The 7-year cumulative incidence of EPI after the first pancreatitis episode was 24%. Genetic risk factors were associated with earlier progression to EPI (HR 1.56; 95% CI 1.02-2.39). CONCLUSIONS: EPI affects nearly 20% of children with ARP/CP. FE is a valuable diagnostic tool in ruling out EPI. Children with genetic risk factors need closer surveillance due to an increased risk for developing EPI.