Inborn errors of metabolism with a protein-restricted diet: Effect on polyunsaturated fatty acids

@article{Sanjurjo1997InbornEO,
  title={Inborn errors of metabolism with a protein-restricted diet: Effect on polyunsaturated fatty acids},
  author={Pablo Sanjurjo and Jos{\'e} Ignacio Ruiz and M. Salcedo Montejo},
  journal={Journal of Inherited Metabolic Disease},
  year={1997},
  volume={20},
  pages={783-789}
}
Previous studies have shown that phenylketonuric patients display a deficiency in long-chain polyunsaturated fatty acids. A study has now been performed on 13 cases (5 with methylmalonic acidaemia and 8 with urea cycle disorders) whose dietary treatment also implies a limitation in protein-rich food. Plasma and red-cell phospholipid fatty acid profiles were studied. The most relevant results were a lower percentage of docosahexaenoic acid in plasma and red-cell phospholipids (0.91% ± 0.53% vs 2… CONTINUE READING
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