1. DIETARY MANAGEMENT OF ISOVALERIC ACIDAEMIA Carmen Yeung TMH Dietitian (APD) yeungkmc@ha.org.hk

2. Dietary Management Aim to limit the dietary leucine intake and minimise formation of isovaleric acid. Sufficient leucine must be given for normal growth requirements

3. How much protein to give? Usually a modest protein restriction 2g/kg in infant, then decrease to 1.0 -1.5g/kg in young children, and combined with adequate energy intake is sufficient to limit the production of isovaleric acid. ( Am J Med Genet C Semin Med Genet, 2006, 142C 95-103) Some reports on treatment with leucine free amino acids and protein intake restricted to below safe levels. Clinical Paediatric Dietetics, 3 rd edition

4. Safe level of protein intake

5. How much protein to give? Usually a modest protein restriction 2g/kg in infant, then decrease to 1.0 -1.5g/kg in young children, and combined with adequate energy intake is sufficient to limit the production of isovaleric acid. Some reports on treatment with leucine free amino acids and protein intake restricted to below safe levels. IMPORTANT: Ensure adequate intake of all vitamins and minerals

6. Treatment of the newly Dx patient May be very sick and in intensive care A protein free feed is given while the infant is stablised, protein is then gradually introduced. (Clinical Paediatric Dietetics, 3 rd edition) Our case, start with protein free formula (PFD-1), and then support with protein contains formula (Similac), the leucine was given according the Nutrition Support Protocols from Ross Metabolic Formula System

7. Recommended intake of leucine

8. Treatment of the newly Dx patient On discharge, our patient is weight at 3.09kg Diet order: Similac 80ml x 4 feeds/d IVA Anamix 80ml x 4 feeds/d Total: 410kcal (= 135kcal/kg/d) 10g protein (=3.23g/kg/d) 444mg leucine (= 148mg/kg/d) Regular Diet FU is essential to monitor the total calories, protein and leucine intake for normal growth

9. Sick Day Diet Aim: reduce production of potentially toxic metabolites from protein catabolism, and provide adequate energy intake (reduced appetite occurs naturally) Estimated energy requirement (EAR) could up to 30% more than usual requirement during acute illness. Adequate hydration Requires frequent feeding, usually Q2-3H

10. Sick Day Diet

11. Long Term Dietary Management Adequate protein intake for growth and development, but not excessive. keep safe level intake of leucine Requirement of ~800mg/d in infancy, gradually up to ~1000-1500mg for normal growth Where is the leucine from?

12. How much of leucine in our food? (Each of the following food contains 50mg leucine) FoodWeight Meat/ Poultry Fish (cooked)2.5g1 oz = 30g Chicken (cooked)2.5g Beef (cooked)2g Egg yolk (cooked)3.6g1 egg yolk = 13g Milk/ Dairy Cows milk15ml Yoghurt10g~ 2 teaspoon Ice-cream15g Milk chocolate5g 1 small cube Cereals Baby Rice cereal (raw)7g~ 1 tablespoon Rice (cooked)25g~ 1 tablespoon Bread10g1/3 slide bread Pasta (cooked)15g Potato (boiled)60g1 egg size Weighing scale, measuring spoons and cups are required for food preparation

13. Meal sample 18 months old girl, BW 12kg FoodCaloriesProteinLeucine BreakfastIVA Anamix 240ml165kcal4.8g0 LunchNoodle ¾ bowl200kcal4g4Ex leucine (200mg) Cooked meat ½ oz40kcal3.5g5Ex leucine (250mg) Vegetable ¼ bowl20kcal0g0 Afternoon tea IVA Anamix 240ml165kcal4.8g0 DinnerRice ¾ bowl200kcal4g4Ex leucine (200mg) Cooked meat ½ oz40kcal3.5g5Ex leucine (250mg) Vegetable ¼ bowl20kcal0g0 SupperIVA Anamix 240ml165kcal4.8g0 TOTAL:1015kcal25.4g protein18Ex Leucine (900mg)

14. How much of leucine in our food? The amount of Leucine can be estimated from the protein content of the food, for example Calculate: 50/ protein in 100g = 50 / 7.4 = 7g baby cereal contains 50mg leucine (= 1 exchange of leucine food) Per 100g baby cereal Energy378kcal Protein7.4g Carbohydrate85g Fat0.9g

15. Summary: Not to over restricted protein intake Monitor leucine intake Reinforced good diet compliance Comply with weighting food items Food label reading Basic calculation of diet