Secondary carnitine deficiency in hyperammonemic attacks of ornithine transcarbamylase deficiency.

@article{Ohtani1988SecondaryCD,
  title={Secondary carnitine deficiency in hyperammonemic attacks of ornithine transcarbamylase deficiency.},
  author={Yoshinobu Ohtani and Kazuhiko Ohyanagi and Shigenori Yamamoto and Ichiro Matsuda},
  journal={The Journal of pediatrics},
  year={1988},
  volume={112 3},
  pages={
          409-14
        }
}
Carnitine status was evaluated in 12 patients with hyperammonemic attacks caused by a deficiency in ornithine transcarbamylase. We found decreased free carnitine and increased acylcarnitine levels in the serum, a decreased free carnitine content and an elevated acyl/free carnitine ratio in the liver, and increased excretion of free and acylcarnitine in the urine. Analyses of urinary acylcarnitine using the secondary ion mass spectrometry technique revealed increased amounts of acetylcarnitine… CONTINUE READING

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