Is Pancreatic Insufficiency in Celiac Disease Due to Transient Enterokinase Deficiency?

Abstract

Purpose: The aim of this study is to determine if the etiology of pancreatic insufficiency (PI) in celiac disease (CD) is due to transient enterokinase deficiency. Methods: This is a prospective study which included patients less than 21 years of age with suspected CD due to abnormal celiac serology who underwent endoscopy between 2009 and 2010. Biopsies were sent for enterokinase and disaccharidase analysis. In addition, secretin stimulation was performed and the duodenal fluid was sent for pancreatic enzyme analysis. Results: A total of 17 patients were identified and 8 patients were diagnosed with Marsh 3a to 3c CD. Signs and symptoms of these patients included failure to thrive, decreased appetite, abdominal pain, vomiting, headaches, constipation, diarrhea, and rashes. All patients had normal enterokinase levels in their duodenal mucosa. Three patients with CD did not have a generalized disaccharidase deficiency. One patient with CD had an isolated amylase deficiency, and one patient had an isolated lipase deficiency. Conclusion: Based on previous literature, the risk of PI appears to be increased in children with CD. In children who have partial or total villous atrophy, it would be expected that they have a generalized disaccharidase deficiency as well as a low enterokinase level, based on the co-localization theory of these brush border enzymes. Surprisingly, all of our patients with 3a to 3c Marsh criteria CD had normal tissue enterokinase levels despite two thirds of the patients having a generalized disaccharidase deficiency. We would expect children with pancreatic enzyme deficiency to have low enterokinase levels, due to the activation cascade of pancreatic digestive zymogens. The 2 patients with isolated PI and CD had normal enterokinase levels. This may suggest enterokinase may not be directly related to PI in CD. Complete PI was not prevalent in our patients with CD. We postulate that complete PI might still be a result of enterokinase deficiency in CD; however, more patients must be enrolled in our study in order to understand the etiology of PI in children with CD.