Claire M. Chee

31P NMR studies in Duchenne muscular dystrophy: Age‐related metabolic changes

To evaluate possible progressive metabolic changes in Duchenne muscular dystrophy, we used 31P nuclear magnetic resonance spectroscopy to measure high-energy phosphate compounds and phosphorylated diesters (PDE) in resting gastrocnemius muscle of 14 Duchenne patients and 10 normal boys. The patients had higher inorganic phosphate (Pi), intracellular pH, and PDE; and lower phosphocreatine (PCr) and PCr/Pi ratio; ATP was not significantly different. The patients showed significant age-related decreases in PCr and PCr/Pi, and increases in Pi and PDE, but ATP did not change. In normal boys, ATP increased with age, but PCr and Pi did not. These studies imply progressive metabolic deterioration in Duchenne dystrophy.

Selenium Deficiency Associated with Cardiomyopathy : A Complication of the Ketogenic Diet

Summary:  Purpose: The ketogenic diet (KD) is an efficacious treatment for intractable epilepsy, associated with infrequent side effects. The KD is known to be deficient in most vitamins and minerals and may be deficient in trace minerals. We report biochemical selenium deficiency in nine patients on the KD, including one who developed cardiomyopathy.

TREATMENT OF ACQUIRED EPILEPTIC APHASIA WITH THE KETOGENIC DIET

Acquired epileptic aphasia remains a poorly understood entity, which is frequently difficult to treat. Previously described treatment modalities have included antiepileptic drugs, corticosteroids, intravenous immunoglobulin, and multiple subpial transections. We describe three patients with acquired epileptic aphasia refractory to traditional treatments who were successfully treated with the ketogenic diet. All three patients had lasting improvement of their language, behavior, and seizures for 26, 24, and 12 months, respectively. This is the first reported series of patients with acquired epileptic aphasia successfully treated with the ketogenic diet, and suggests a new therapeutic alternative for patients with this often difficult-to-treat disorder. (J Child Neurol 1999;14:696-701).

Reconciling diabetes management and the ketogenic diet in a child with 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 < 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).

Manipulation of types of fats and cholesterol intake can successfully improve the lipid profile while maintaining the efficacy of the ketogenic diet.

The ketogenic diet (KD) is a dietary therapy that effectively treats intractable epilepsy in children. Hyperlipidemias are common side effects of KD and have been reported in approximately 30% to 50% of patients on this diet. Fats comprise 90% of the calories, and saturated fats are often used as 50% of the fat intake. The purpose of this study was to investigate whether alterations of the dietary fat sources can improve the lipid profiles while maintaining seizure control in the ketogenic diet. Using a retrospective chart review, the authors evaluated their practices of treating hyperlipidemias in 6 children on the KD who developed elevated lipid profiles. All had normal or mildly elevated lipid profiles before beginning the KD. The elevated lipid levels were detected after an average of 7 months of KD treatment. Changing the dietary fat types to mainly oils (polyunsaturated fats) and decreasing the use of protein foods high in saturated fats and cholesterol resulted in a normalized or baseline lipid pro...

Epilepsy Treatment Simplified through Mobile Ketogenic Diet Planning

BACKGROUND The Ketogenic Diet (KD) is an effective, alternative treatment for refractory epilepsy. This high fat, low protein and carbohydrate diet mimics the metabolic and hormonal changes that are associated with fasting. AIMS To maximize the effectiveness of the KD, each meal is precisely planned, calculated, and weighed to within 0.1 gram for the average three-year duration of treatment. Managing the KD is time-consuming and may deter caretakers and patients from pursuing or continuing this treatment. Thus, we investigated methods of planning KD faster and making the process more portable through mobile applications. METHODS Nutritional data was gathered from the United States Department of Agriculture (USDA) Nutrient Database. User selected foods are converted into linear equations with n variables and three constraints: prescribed fat content, prescribed protein content, and prescribed carbohydrate content. Techniques are applied to derive the solutions to the underdetermined system depending on the number of foods chosen. RESULTS The method was implemented on an iOS device and tested with varieties of foods and different number of foods selected. With each case, the applications constructed meal plan was within 95% precision of the KD requirements. CONCLUSION In this study, we attempt to reduce the time needed to calculate a meal by automating the computation of the KD via a linear algebra model. We improve upon previous KD calculators by offering optimal suggestions and incorporating the USDA database. We believe this mobile application will help make the KD and other dietary treatment preparations less time consuming and more convenient.

Ketogenic Diet Patients’ Lipid Profiles Improved With KetoCal 4:1 Liquid A Case Series

Objective. The ketogenic diet (KD) can result in hyperlipidemias. We report improvements in the lipid profiles of 3 children who were switched from KetoCal 4:1 powder (formulation prior to fall 201...

Use of Expressed Breast Milk With the Ketogenic Diet

Breastfeeding continues to be the recommended mode of infant feeding with numerous nutritive and nonnutritive benefits to both mothers and babies. The ketogenic diet (KD) is a high-fat, adequate pr...