Abstract
Eleven children with cystic fibrosis (CF) and pancreatic insufficiency were given supplementation with taurine (30–40 mg/kg/day) for 2 months, while taking their usual dosage of enzymatic therapy. One patient dropped out of the study because she developed severe constipation. In the other 10 patients, urinary taurine excretion (88 ± 30.1 mg/m2s.a./24 h) was similar to that of controls (86.2 ± 6 mg/m2s.a./24 h) before taurine and increased markedly after supplementation (618.2 ± 79.97 mg/m2s.a./24 h), indicating efficient intestinal absorption. Their coefficient of fat absorption was 81.2 ±2.3% and increased significantly after taurine (91.3 ±1.13%; p < 0.01); the area under the curve of plasma triglyceride postprandial levels (1 ± 0.1 mg × min/ml) also increased significantly after taurine (1.4 ± 0.3 mg × min/ml; p < 0.05), showing values very similar to those of controls. Conversely, no change was observed in the serum postprandial levels of glycocholic acid: the maximum postprandial peak before (1.2 ± 0.3 μmol/1) and after taurine (1 ± 0.1 μmol/1) remained significantly lower than in controls (2.4 ± 0.3 μmol/1); p < 0.01 and p < 0.001, respectively. Mean total fecal bile acid (BA) excretion was 10.24 ±2.15 mg/kg/day before taurine and 12.8 ± 4.27 mg/kg/day after taurine (normal pediatric values, 2.91 ± 1.1 mg/kg/day); however, in the individual patients we found a variable trend, four of them showing a net increase in fecal BA excretion. We conclude that taurine supplementation is confirmed as a useful form of adjuvant therapy in CF patients with severe pancreatic insufficiency, probably as a result of an improvement in the intraluminal phase of fat digestion. However, more work is necessary to define better its effect on BA metabolism in these patients.