Reconciling diabetes management and the ketogenic diet in a child with pyruvate dehydrogenase deficiency.

[pyruvate dehydrogenase deficiency]

A 4 -year -old girl with pyruvate dehydrogenase deficiency , static encephalopathy , and seizure disorder treated with the ketogenic diet presented in severe diabetic ketoacidosis . Pyruvate dehydrogenase deficiency is a rare genetic defect of mitochondrial energy metabolism that leads to inefficient glucose use and lactic acidosis . The ketogenic diet provides the brain with an alternate fuel source , but its implementation opposes traditional diabetes management . Faced with this therapeutic dilemma , we aimed to maintain ketosis without compromising safety to optimize neurologic function and quality of life . This is the first report , to our knowledge , of a child simultaneously treated with the ketogenic diet and exogenous insulin . A 28 -month follow-up revealed excellent glycemic control , improved activity level , significant developmental achievements , and , perhaps most striking , catch-up linear growth from < 5 th percentile to the 50 th percentile . Her progress to date indicates that diabetes does not preclude use of the ketogenic diet .

Diseases
Validation

Diseases presenting "dehydrogenase deficiency" symptom

adrenomyeloneuropathy

krabbe disease

pyruvate dehydrogenase deficiency

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