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WorksheetsCA MNT INBORN ERROR METABOLISM
Total questions: 35
Worksheet time: 2hrs 40mins
Medical management target for PKU is ___________________
To maintain Phe 1-6 mg/dl
To maintain Tyr 1-6 mg/dl
To maintain phenylalanine hydroxylase (PAH) 1-6 mg/dl
To maintain Phe: Tyr ratio Phe >6-10 mg/dL: Tyr <3 mg/dL
List of essential amino acids except....
Isoleucine
Lysine
Phenylalanine
Alanine
One of the Principle of MNT for PKU
Phe.-free formula/ medical foods.
Lysine supplements
Low potassium foods
Increase intake of phenylalanine foods
3 branched-chain amino acids that involved in Maple syrup urine disease (MSUD) are
alanine, arginine & glycine
leucine, isoleucine, & valine
leucine, isoleucine, & cysteine
leucine, isoleucine, & lysine
The dietary managements for MSUD...
A diet restricted in lysine, tryptophan, and phenyalanine is used
Commercial formula mix without the BCAA eg protein-free diet powder, small amounts of milk and a protein free supplement has been described.
Fruits, vegetables, and cereals high in protein is prescribed
Use of standard infant/children milk formulas
Nutrition therapy for urea cycle disorders
Low protein
High protein
Moderate protein
avoid protein 100%
All are formulas for Urea Cycle Disorders (UCD) except
The taste of amino acids is
sweet
bitter
sour
umami
Galactosemia is caused by the absence of an enzyme ______________________ which is needed in the liver for the conversion of galactose to glucose.
enzyme galactose-1-phosphate
enzyme galactokinase-1-phosphate uridyl transferase,
enzyme galactose-1-phosphate uridyl transferase
enzyme galactose-1-phosphate uridyl transkinase
Milk substitutes suitable for galactosemia cases are:
Fruits that was not allowed for galactosemia patient to eat due to higher content of galactose [ >10 mg galactose/100grams] are
dates, papaya & watermelon.
mango, kiwi & apple
starfruit & durian
rambutan, orange and banana
GOALS MNT OF GLYCOGEN STORAGE DISEASES
ARE EXCEPT...
PREVENT HYPOGLYCEMIA
CHO: 60-70% OF TOTAL CALORIE INTAKE
PROTEIN : LEAN SOURCES 20% OF TOTAL CALORIE INTAKE.
FAT: 30% OF TOTAL KCAL AND DIETARY SFA SHOULD BE LIMITED.
What is the management for Intermittent MSUD?
Oral feedings can be started after fluid and electrolyte imbalances are corrected.
Provide with peritoneal or hemodialysis to remove alpha ketoacids.
Dietary restriction of lactose and galactose.
Thiamine supplementation and protein restricted diet.
Example of Free Protein Products EXCEPT
carborie
Polycose
Myotein
MCT Oil
One of the MNT for PKU
To restricts the intake of alanine.
To keep phenylalanine levels within desirable range.
To restricts the intake of arginine.
To keep isoleucine levels within desirable range.
What is the task expected of children with PKU by age level of 2-3 years?
Distinguished between "yes" and "no" foods.
Counting how many
Measuring how much
writing basic notes in food diary
L-Tyrosine supplementation is needed to maintain the tyrosine intake at _____________ during pregnancy.
20 mg/kg/day
50 mg/kg/day
110 mg/kg/day
150 mg/kg/day
Clinical changes for infants/children with MSUD
Regular respiration
Hyperglycemia
Able to suck satisfactorily
Seizures may occur
In organizing a low protein diet in UCD cases, the osmolality of the formula must not be __________.
> 420 mOsm/l.
> 200 mOsm/l.
> 300 mOsm/l.
> 400 mOsm/l.
Products containing free amino acids taste best when __________________________
they are served very hot.
they are served very cold.
they are served with sugar.
they are served with carborie.
Complete the following:
Glucose + _________ = Lactose
glucose
fructose
galactose
maltose
Foods allowed for patient diagnosed with galactosemia except....
egg
nuts
cereals
animal milk
The distribution of total calorie intake for GSD 1 :
2/3 DAILY CALORIE INTAKE- DAYTIME AND 1/3 – OVERNIGHT.
1/3 DAILY CALORIE INTAKE- DAYTIME AND 2/3 – OVERNIGHT.
1/2 DAILY CALORIE INTAKE- DAYTIME AND 1/2 – OVERNIGHT.
same as healthy individuals
Please calculate the concentration of formula given below:
Cyclinex 1
100 gram = 510 kcal
1 tbs = 8 gram
Mrs. A prepare 3.5 tbs of Cyclinex 1 powder with 150 ml of water.
What is the concentration of feeding?
3.4 kcal/ml
0.3 kcal/ml
0.67 kcal/ml
0.95 kcal/ml
Boy A
14 months
Male
Energy Requirement - 1200 kcal/day
protein requirement of Boy A was 23 gram per day.
By using the steps in organizing a low protein diet in managing UCD patient, please calculate the remaining protein needed for Boy A.
9 gram protein
6.9 gram protein
7.4 gram protein
23 gram protein
Name the enzyme missing/inactive in PKU
Phe hydroxylase
Tyr hydroxylase
Phe hydroxylanine
Tyr hydroxylanine
In general, the nutrition treatment for Galactosemia________
Eliminate galactose
Eliminate fructose, maltose
Eliminate sucrose, glucose
Eliminate lactose, galactose
The incidence of MSUD
MSUD : 1/10,000 to 1/25,000 live birth
no data
MSUD : 1/100,000 to 1/300,000 live birth
MSUD : 1/15,000 to 1/35,000 live birth
What happened when tyrosine formation is blocked?
production of melanin is decreased
production of melanin is increased
production of urea is decreased
production of urea is increased
Phe > 20 mg/dL is predictor of _____
allergic to protein
increase sensitivity of pain
IQ improvement
IQ loss
Nutramigen
Is a soy based formula
Prescribed for patient with PKU
Made from casein hydrolysate
Is one of the modular product
What is the estimated calories for 1 scoop of carborie if you are planning a diet using it?
1 scoop = 2 gram
30 kcal
8 kcal
2 kcal
150 kcal
ALL listed here may contain phenylalanine except
carob
nutrasweet
hydrolysed protein
polycose
What is the nutrition treatment for Arginase deficiency?
Low protein , L-tyrosine
Low protein, L-amino acids
Low phenyalanine, L-tyrosine
High protein, Low lactose
Please calculate the total protein in foods listed below:
2 slices of bread
+ 1/2 glass of full cream milk
=________________________g protein
4
8
10
12
