Novel intravenous (13)C-methionine breath test as a measure of liver function in children with short bowel syndrome.

摘要

Monitoring hepatic function in children with short bowel syndrome (SBS) and parenteral nutrition-associated liver disease (PNALD) is currently limited to conventional blood testing or liver biopsy. Metabolism of the stable isotope L[1-(13)C]methionine occurs exclusively in liver mitochondria and can be noninvasively quantified in expired breath samples. We hypothesized that the (13)C-methionine breath test ((13)C-MBT) could be a safe, noninvasive, and valid measure of hepatic mitochondrial function in children with SBS and PNALD. METHODS: Baseline breath samples were collected in 8 children with SBS before intravenous administration of 2 mg/kg of L[1-(13)C]methionine. Six paired breath samples were obtained at 20-minute intervals. The (13)CO(2) enrichment was analyzed using isotope ratio mass spectrometry. RESULTS: All 8 patients (5 males; mean age, 8.9 months) tolerated the (13)C-MBT without adverse events. Two patients underwent serial testing. One patient, tested before and after resolution of cholestasi