Monitoring pancreatin supplementation in cystic fibrosis patients with the 13C-Hiolein breath test: evidence for normalized fat assimilation with high dose pancreatin therapy.

Background13C-Hiolein is a randomly 13C-labeled mixture of long chain triglycerides synthesized by algae.MethodsBecause the 13C-Hiolein breath test is a suitable noninvasive tool to detect and monitor pancreatic steatorrhea, we used this new breath test for monitoring the effect of enzyme replacement therapy with an acid resistant enteric coated polydisperse pancreatin preparation (1.500 U/kg d) in children with cystic fibrosis.ResultsAdministration of 1.5 mg/kg 13-C-Hiolein together with a physiological mixed meal (1.5 g/kg rice cookies, containing 25% fat and 37% starch) resulted in significantly higher breath 13CO2/12CO2 ratios in controls than in cystic fibrosis children (maximal delta over baseline responses (DOBmax) 39.2 +/- 18.1% vs. 13.1 +/-13.9%; p < 0.001). With pancreatin, DOBmax in the cystic fibrosis patients responses returned completely to normal (39.2 +/- 29.2% DOBmax). A breath hydrogen increase indicating the malassimilation of starch was noticed in one patient with severe pancreatic insufficiency only.ConclusionIn contrast to fecal fat analysis, the 13C-Hiolein breath test reflects postprandial fat assimilation immediately after a given, labeled meal. Monitoring the oxidative fate of physiological test meal with a stable isotope breath test, this study shows that fat assimilation in cystic fibrosis patients can be normalized with high dose pancreatin.