title: Effect of supplementation with L-carnitine at a small dose on acylcarnitine profiles in serum and urine and the renal handling of acylcarnitines in a patient with multiple acyl-coenzyme A dehydrogenation defect.
abstract: We studied the effects of L-carnitine supplementation at a small dose on the profiles of acylcarnitines in serum and urine, as well as the renal handling of acylcarnitines, in a patient with multiple acyl-coenzyme A dehydrogenation defect. After supplementation with L-carnitine at a dose of 20 mg/kg/day, the concentration of each acylcarnitine measured both in the serum and in the urine had increased significantly, with the exception of that of an acylcarnitine with a carbon chain length (C) of 8 (C8 acylcarnitine). The magnitude of increase in the concentrations of the acylcarnitines in the serum was not associated with chain length, whereas in the urine, the magnitude tended to be greater in proportion to the shortness of the chain length. The fractional excretions of C2-C5 acylcarnitines exceeded 100%, indicating that they were produced in, or transported across, renal tubular epithelial cells and secreted into the urine. These results indicate that supplementation with a relatively small amount of L-carnitine can enhance the renal excretion of accumulated short-chain-length acylcarnitines through tubular excretion, in addition to basic glomerular filtration.
authors.affiliation: Department of Pediatrics and Child Health, Kurume University School of Medicine, 67 Asahi-machi, Kurume 830-0011, Japan. yoshino@med.kurume-u.ac.jp
authors.forename: Makoto
authors.initials: M
authors.lastname: Yoshino
authors.forename: Yasuykuki
authors.initials: Y
authors.lastname: Tokunaga
authors.forename: Yoriko
authors.initials: Y
authors.lastname: Watanabe
authors.forename: Ichiro
authors.initials: I
authors.lastname: Yoshida
authors.forename: Miki
authors.initials: M
authors.lastname: Sakaguchi
authors.forename: Ikue
authors.initials: I
authors.lastname: Hata
authors.forename: Yosuke
authors.initials: Y
authors.lastname: Shigematsu
authors.forename: Masahiko
authors.initials: M
authors.lastname: Kimura
authors.forename: Seiji
authors.initials: S
authors.lastname: Yamaguchi
mesh_terms: D042964:Acyl-CoA Dehydrogenase / Q000378:metabolism*; D002331:Carnitine / Q000008:administration & dosage* / Q000031:analogs & derivatives* / Q000097:blood / Q000378:metabolism* / Q000652:urine; D006801:Humans; D007231:Infant, Newborn; D007668:Kidney / Q000503:physiopathology*; D008297:Male; D008661:Metabolism, Inborn Errors / Q000097:blood / Q000378:metabolism* / Q000652:urine; D021241:Spectrometry, Mass, Electrospray Ionization
chemical_list: C116917:acylcarnitine; D042964:Acyl-CoA Dehydrogenase; D002331:Carnitine