Embed Size (px)
Citation preview
Dietary Management
Aim to limit the dietary leucine intake and minimise formation of isovaleric acid.
Sufficient leucine must be given for normal growth requirements
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, 3rd edition
Safe level of protein intake
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
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, 3rd 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
Recommended intake of leucine
Treatment of the newly Dx patient
On discharge, our patient is weight at 3.09kg
Diet order:Similac ≤ 80ml x 4 feeds/dIVA Anamix ≥80ml x 4 feeds/dTotal: 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
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
Sick Day Diet
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?
How much of leucine in our food?(Each of the following food contains 50mg leucine)
Food Weight
Meat/ Poultry
Fish (cooked) 2.5g 1 oz = 30g Chicken (cooked) 2.5g
Beef (cooked) 2g
Egg yolk (cooked) 3.6g 1 egg yolk = 13g
Milk/ Dairy
Cow’s milk 15ml
Yoghurt 10g ~ 2 teaspoon
Ice-cream 15g
Milk chocolate 5g 1 small cube
Cereals
Baby Rice cereal (raw) 7g ~ 1 tablespoon
Rice (cooked) 25g ~ 1 tablespoon
Bread 10g 1/3 slide bread
Pasta (cooked) 15g
Potato (boiled) 60g 1 egg size
Weighing scale, measuring spoons and cups are required for food preparation
Meal sample18 months old girl, BW 12kg
Food Calories
Protein Leucine
Breakfast
IVA Anamix 240ml
165kcal
4.8g 0
Lunch Noodle ¾ bowl 200kcal
4g 4Ex leucine (200mg)
Cooked meat ½ oz
40kcal 3.5g 5Ex leucine (250mg)
Vegetable ¼ bowl
20kcal 0g 0
Afternoon tea
IVA Anamix 240ml
165kcal
4.8g 0
Dinner Rice ¾ bowl 200kcal
4g 4Ex leucine (200mg)
Cooked meat ½ oz
40kcal 3.5g 5Ex leucine (250mg)
Vegetable ¼ bowl
20kcal 0g 0
Supper IVA Anamix 240ml
165kcal
4.8g 0
TOTAL: 1015kcal
25.4g protein
18Ex Leucine (900mg)
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
Energy 378kcal
Protein 7.4g
Carbohydrate 85g
Fat 0.9g
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
