• Causes a buildup of pyruvate that gets shunted to lactate (via LDH) and alanine (via ALT).
• X-linked.
• Findings
  • Neurologic defects, lactic acidosis, ↑ serum alanine starting in infancy.
• Treatment
  • ↑ intake of ketogenic nutrients (eg, high fat content or ↑ lysine and leucine).