2021 Volume 57 Issue 1 Pages 135-139
The carnitine kinetics of three extremely low birth weight infants who received sivelestat sodium（sivelestat） for severe respiratory distress were investigated. Free carnitine（C0）is consumed during the excretion process of the pivoxyl group present on sivelestat, while pivaloylcarnitine（C5piv）is synthesized and excreted in the urine. C0 was found to decrease with administration of sivelestat, and to remain low for about 2 weeks post administration. C5piv, on the other hand, was found to be elevated in both blood and urine following sivelestat administration while urinary excretion continued for approximately 2 weeks. The effect of sivelestat on carnitine metabolism has previously been mentioned and our results support these findings. It has been suggested that extremely low birth weight infants are more likely to become carnitine deficient and require carnitine supplementation, not only during sivelestat administration, but also afterwards. In addition, the increase of C5piv in blood after sivelestat administration may cause false positive isovaleric acidemia in neonatal mass screening and caution is therefore required.
