Dietary Management of Isovaleric Acidemia

Report
DIETARY MANAGEMENT OF
ISOVALERIC ACIDAEMIA
Carmen Yeung
TMH Dietitian (APD)
[email protected]
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/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
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
Chicken (cooked)
2.5g
Beef (cooked)
2g
Egg yolk (cooked)
3.6g
1 oz = 30g
1 egg yolk = 13g
Milk/ Dairy
Cow’s milk
15ml
Yoghurt
10g
Ice-cream
15g
Milk chocolate
5g
1 small cube
Baby Rice cereal (raw)
7g
~ 1 tablespoon
Rice (cooked)
25g
~ 1 tablespoon
Bread
10g
1/3 slide bread
Pasta (cooked)
15g
Potato (boiled)
60g
~ 2 teaspoon
Cereals
1 egg size
Weighing scale, measuring spoons and cups are required for food preparation
Meal sample
18 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
IVA Anamix 240ml
165kcal
4.8g
0
Supper
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
Per 100g baby cereal

Energy
378kcal
Protein
7.4g
Carbohydrate
85g
Fat
0.9g
Calculate:
50/ protein in 100g
= 50 / 7.4
= 7g baby cereal contains 50mg leucine (= 1 exchange
of leucine food)
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

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