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Normal or low lactate/pyruvate ratio (<25) and increased pyruvate concentration occurs in defects of pyruvate dehydrogenase (not associated with hypoglycemia), glucogenic enzymes deficiencies (associated with hypoglycemia), and partial pyruvate carboxylase deficiency (this form of pyruvate carboxylase deficiency usually does not occur in neonates). High lactate/pyruvate ratio (usually >35) and decreased or normal pyruvate occurs with total pyruvate carboxylase deficiency (the usual form that presents in neonates) or respiratory chain defects. Respiratory chain defects are associated with an increased ratio (normally 2:1 or less) of 3-hydroxybutyrate to acetoacetate. More about... 45


Evaluation and treatment of a comatose neonate must be carried out simultaneously in order to prevent further brain damage. Further brain damage is avoided by preventing and correcting systemic and neurological causes of secondary brain damage while simultaneously treating the primary insult if it is still present.


Oxygen by hood, mechanical ventilation, or extracorporeal membrane oxygenation should be used to keep arterial oxygen saturation above 95% or arterial pO2 above 70 torr. Levels of pCO2 should be kept between 35 to 45 mm Hg. Mean blood pressure should be kept at about 50 mm Hg. Serum blood sugar should be kept above 75 mg/dL. Respiratory acidosis is corrected by increasing the positive end expiratory pressure or the ventilatory rate. Metabolic acidosis should be corrected when arterial base deficit exceeds 7 mEq/L. Sodium bicarbonate should be used at a dose of 2 to 5 mEq/kg over 5 to 10 minutes and repeated if necessary to maintain blood pH above 7.20. Hemodialysis is an effective method of correcting persistent metabolic acidosis.


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