Congenital lactic acidosis.

  title={Congenital lactic acidosis.},
  author={Yasuo Kuroda and Etsuo Naito and Eiji Takeda and Ichiro Yokota and Masuhide Miyao},
  volume={38 1-4},
For use in screening for disorders of pyruvate metabolism in cultured skin fibroblasts, we developed a sensitive assay method for measuring the rates of decarboxylation of (1-14C)-pyruvate using dichloroacetate (DCA). By this method, disorders of the mechanism of activation of pyruvate dehydrogenase (PDH) were found in 2 of 10 patients with congenital lactic acidosis of unknown cause. We also found that DCA activated the PDH complex and the tricarboxylic acid cycle, which is an important… CONTINUE READING