2018/10/20. Use of cooking oils in the classical ketogenic diet for intractable pediatric epilepsy. Disclosures. Outlines

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1 // Disclosures Use of cooking oils in the classical ketogenic diet for intractable pediatric epilepsy Dr. Hsiu-Fen Lee has no other relevant affiliation or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the presentation apart from those disclosed Hsiu-Fen Lee, M.D. Ph.D. Division of Pediatric Neurology, Children s Medical Center, Taichung Veterans General Hospital, Taichung, Taiwan Outlines Introduction Use of cooking oils in a : ratio classical ketogenic diet for intractable pediatric epilepsy Long-term effectiveness and tolerability Take home messages Introduction Chronological changes of oils used in the ketogenic diet in TPVGH Year Oil 9-9 Saturated fat MCT oil MCT oil After Cooking oils Use of cooking oils in a : ratio classical ketogenic diet. Exclude contraindications for the ketogenic diet. A clinical pathway for the ketogenic diet. A Meal plan Wahn JR, Chi CS et al. J Chin Nutrition Soc 99;9:9-7 Mak SC, Chi CS et al. Acta Paediatr Sin 999;:97-

2 // Contraindication for the ketogenic diet Exclusion of physical illness Absolute Carnitine deficiency (primary) Carnitine palmitoyltransferase (CPT) I or II deficiency Carnitine translocase deficiency β-oxidation defects Medium-chain acyl dehydrogenase deficiency (MCAD) Long-chain acyl dehydrogenase deficiency (LCAD) Short-chain acyl dehydrogenase deficiency (SCAD) Long-chain -hydroxyacyl-coa deficiency Medium-chain -hydroxyacyl-coa deficiency Pyruvate carboxylase deficiency Porphyria Relative Inability to maintain adequate nutrition Surgical focus identified by neuroimaging and video- EEG monitoring Parent or caregiver noncompliance Propofol concurrent use (risk of propofol infusion syndrome may be higher) Liver or renal function impairment Renal calculi Dyslipidemia Kossoff EH et al., Epilepsia Open ;:7-9 Day : Hospital admission for the KD A clinical pathway for the ketogenic diet Review seizure history and the AEDs prescriptions Consult dietitian Fluids restricted to 7 to % of maintenance water ( cc/day) Fasting starts in the midnight except for water and AEDs Monitor S/S of hypoglycemia and record seizure frequency A : ratio classical ketogenic diet Total daily calorie Day : Biochemical surveys Fasting continues Laboratory survey in the morning include CBC, renal & liver functions, arterial blood gas, blood sugar, blood ketone, fasting lipid profiles, and urinalysis of ketone and Ca/Cr ratio Blood sugar and urine ketones monitored every h until urine ketones + to + Monitor S/S of hypoglycemia and record seizure frequency F : ( P + C ) = : % % % Days -: The diet initiation Given a half (D), three quarters (D), full (D) of calculated diet meal, and divided equally into breakfast, lunch, and dinner Parents check urine ketones every morning and record seizure frequency Monitor the adverse and positive effects, and the compliance of the diet Multivitamin with minerals, iron, and trace elements and calcium supplements Educational program completed, including home preparation of the diet and the use of urine strip Fa t Protein Carbhydrates Age Meal plan - Total Daily Calorie Male Total Daily Calorie needs (Kcal), Dietary Reference Intakes (DRIs) Female - months /kg /kg 7- months 9/kg 9/kg - years (Slightly low) (Appropriate) - years (Slightly low) (Appropriate) 7-9 years (Slightly low) (Appropriate) - years (Slightly low) (Appropriate) - years (Slightly low) (Appropriate) - years (Low) (Slightly low) 9 (Appropriate) (High) (Slightly low) (Appropriate) (Slightly low) (Appropriate) (Slightly low) 9 (Appropriate) 9 (Slightly low) (Appropriate) (Slightly low) (Appropriate) (Low) 9 (Slightly low) (Appropriate) (High) Health Promotion Administration, Ministry of Health and Welfare, Taiwan Search Calculate For example, total daily calorie needs Kcal Database of food composition in Taiwan Weight (g) Fat (g) Protein (g) Carbohydrate(g) Rice..9. Pig hind legs meat.. Egg... Vegetable oil Total(g) Kcal % * For infants with liquid ketogenic diet, carbohydrate and protein intakes are replaced by malt sugar and protein powder, respectively.

3 // Saturated Cooking oils Monounsaturated Polyunsaturated Olive oil Camellia oil...9. Linseed oil Perilla oil Grape seed oil Lard Linolenic acid (%) Sample ketogenic diet recipes Date Sun Mon Tue Wed Thu Fri Sat Breakfast Lunch Dessert Dinner Omelet Mushroom Braised beef with Fried fish with mushroom Meat Blueberry chocolate cake Barley tea (no Stewed beef rib Fried shrimp Steamed fish Fried arden lettuce Beef with Fried broccoli Scrambled egg with carrot Porridge Rib and radish Sliced pork with mashed garlic Braised tofu with shrimp Radish and sliced pork Cupcakes Barley tea (no Steamed whitemeat fish and avocado Creamy cabbage with pork neck Pork Rib and radish Chicken burns Soybean milk (no Pork bone broth Braised pork with bamboo strip Egg with tofu Fried amaranth Tofu Scallion pancake Black tea (no Braised pork balls Fried milkfish belly Fried water spinach Pork belly balls Fried bok choy Scrambled egg with Pork Ribs and sweet corn Soup Crispy chicken steak Fried Zucchini Scrambled egg with cheese White gourd Souffle cake roll Green tea (no Minced pork with eggplant Avocado salmon Screamed eggs with cream Fried broccoli Chicken with mushroom Ham & egg bun Soy milk (no Tofu Fried milkfish Soy sauce braised tofu Fried loofah Golden mushroom& corn Mixed pizza Wheat tea (no Braised chicken leg John Dory fish with lemon Fried mushroom with butter Fried arden lettuce Laminaria japonica and egg Fried water spinach Over-hard egg Assorted vegetables Curry chicken Fried celery and dried bean curd Fried water spinach Bamboo shoots Chiffon cake Organic herbal tea Oster omelet Perch with white cauliflower Seafood salad Fried Komatsuna Sliced meat and whole wheat toast Soy milk (no Pork bone broth Steamed minced pork with pickles and mushrooms Fried salmon belly Fried lettuce Pork Ribs and sweet corn Soup Cheesecake Organic Matcha Duck confit Fried celery with pork and seafood Mushrooms and white cauliflower Fried spinach Pork rib and white gourd Menu Menu Lunch g Broccoli g Capsicum annuumand chicken g Fried mushrooms with butter 9 g Fried eggs with dried radish and Arugula g Rice g Four-Herbal Soup (with perilla oil) Dinner g Broccoli g Fried water spinach g Stewed beef with g Fried milkfish belly g Rice g Clams and winter gourd (with perilla oil) Scallion pancake Batter g Mozzarella cheese g Yogurt (no One egg ¼ cup Linseed powder ¼ cup Gluten-free oat bran ½ cup Organic raw soybean flour tea spoon Psyllium powder tea spoon Baking powder ¼ tea spoon Sea salt g Scallions Olive oil Curst Eggs g Cream cheese g Erythritol cm Vanilla planifolia g Lemon juice Stuffing g Fresh cream g Erythritol cm Vanilla planifolia Soufflé cake roll Desserts Scallion pancake Mixed pizza Creamy chicken mushroom pie Currypuff Chocolate almond cake Long-term effectiveness and tolerability Cupcakes Soufflé cake roll Chiffon cake Cheesecake Moon cakes

4 // Baseline demographics Etiologies of epilepsy From Apr. to Apr., subjects who adhered to the diet for at least months were included Seizure-related demographics N = Gender, Male: Female 9: Age at first seizure, median (range) mo ( days 7 years 9months) Classification of seizure types Focal seizure with or without evolving into 9 (9%) bilateral tonic-clonic seizures, n(%) Generalized seizures, n(%) (%) Numbers of AED prescriptions before the diet, median (range) (-) Diet-related demographics Age at diet commencement, median (range) years months ( months - 7 years 9 months) Duration on the diet, median (range) year months ( months years months) Lee HF et al., Epilepsy Res ;7:7-79 7% % % % Ge netic Structural Metabolic Unk now n Genetic disorders: Dravet syndrome (n=), CDKL (n=), Angelman syndrome (n=), KCNQ (n=), KCNT (n=), Chromosomal disorder (n=) Structural disorders: cortical malformation (n=), postinfectious (n=), posttraumatic (n=) Metabolic disorders: Pyruvate dehydrogenase complex deficiency (n=) Lee HF et al., Epilepsy Res ;7:7-79 Epilepsy syndromes Age distribution and feeding methods of patients who received the diet % 7 7% 7 7 FIRES ES MAE DS EIMFS EIEE NS SD 9/ (%) of subjects were specific epilepsy syndromes FIRES, febrile infection related epilepsy syndrome; ES, epileptic spasms; MAE, myoclonic atonic epilepsy; DS, Dravet syndrome; EIMFS, Epilepsy of infancy with migrating focal seizures; EIEE, early infantile epileptic encephalopathy; NSSD, no specific syndrome diagnosis % % % < year to < years to < years to years % % Orally NG tube Gastrostomy tube Lee HF et al., Epilepsy Res ;7:7-79 Lee HF et al., Epilepsy Res ;7:7-79 Clinical outcomes Numbers of AED prescriptions after the KD (%) % % (7%) % (%) (%) % % (7%) 7% % 7% 7% % % % % % % % % % % % months (*%) months (*%) months (*%) months (*%) > years (*%) % > 9 % -9 % < % - No. adhering to the diet * Responders, seizure reduction % ** subjects were seizure-free, including myoclonic-atonic epilepsy (n=), epileptic spasms (n=), Angelman syndrome (n=), focal cortical malformation (n=), post-traumatic epilepsy (n=), pyruvate dehydrogenase complex deficiency (n=), and unknown causes (n=) Lee HF et al., Epilepsy Res ;7:7-79 7% % % No change Decreased Dis continuation Lee HF et al., Epilepsy Res ;7:7-79

5 // Adverse effects The reasons of cessation of the ketogenic diet Gastrointestinal discomfort, n = (/; %) Hyperlipidemia, n = (/; 9%) 7 % Constipation Dia rrhea Vomitng Abdominal floating Hyper-TG Hyper-Chol Hyper-TG + Chol 7 % % % % No improve me nt in seizure Se iz ure -fre ed om a ch ie ved Fo od refu sal Adv erse effects Poor parentalcompliance co ntro l Lee HF et al., Epilepsy Res ;7:7-79 Lee HF et al., Epilepsy Res ;7:7-79 Take home messages The : classical KD was shown to be effective at short- and long-term follow-up. Seizure-free status was observed in % of subjects at months and at > years. A greater than % seizure reduction was found in % and % of subjects at months and at > years, respectively. The most common reason for diet cessation was no improvement in seizure control. The : classical KD was shown to be safe and well tolerated in pediatric epilepsy. Thank you for your time