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Reconciling Diabetes Management and the Ketogenic Diet in a Child with Pyruvate Dehydrogenase Deficiency

Division of Pediatric Endocrinology, Columbus Children's Hospital Columbus, Ohio

Paul S. Thornton, MD

Division of Endocrinology Cook, Children's Hospital Fort Worth, Texas

Christina M. Preis, MSN, CRNP

Division of Endocrinology and Diabetes, The Children's Hospital of Philadelphia Philadelphia, Pennsylvania

Claire Chee, RN, BS

Division of Neurology, The Children's Hospital of Philadelphia Philadelphia, Pennsylvania

Adda Grimberg, MD, FAAP

Division of Endocrinology and Diabetes, The Children's Hospital of Philadelphia Philadelphia, Pennsylvania, grimberg@email. chop. edu

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 < 5th percentile to the 50th percentile. Her progress to date indicates that diabetes does not preclude use of the ketogenic diet. (J Child Neurol 2006;21:436—439; DOI 10.2310/7010.2006.00109).

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