Glutaric Aciduria ‘type Ii’

  title={Glutaric Aciduria ‘type Ii’},
  author={Hildegard Przyrembel and Udo Wendel and Sybe K. Wadman and Hans J. Bremer},
  journal={Pediatric Research},
A new fetal metabolic disorder is described, mainly characterised by severe acidosis and hypoglycaemia, a' sweaty-feet' like odour, and massive glutaric aciduria and acidaemia. In addition, urinary excretion of isobutyric and isovaleric acid was increased, as well as that of some dicarboxylic acids. Serum levels of various long-chain and short-chain fatty acids and dicarboxylic acids and plasma levels of valine and lysine were markedly elevated. The degradation of 14C-labelled glutaric acid… CONTINUE READING