Follow-up of a child with pyruvate dehydrogenase deficiency on a less restrictive ketogenic diet.

[pyruvate dehydrogenase deficiency]

A male child with X-linked pyruvate dehydrogenase deficiency presented with severe neonatal lactic acidosis . Poor compliance following initiation of the ketogenic diet justified modification to a less restrictive form which improved compliance . One year after starting the modified diet , he remained clinically stable , showing developmental progress .