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Inborn Error of Metabolism

Total questions: 20

Worksheet time: 10mins

Name
Class
Date
1.

Overflow type of Aminoacidurias

a)

PKU

b)

Faconi's syndrome

c)

Cystinuria

d)

Tyrosinuria

2.

It is capable of screening infant blood sample for specific substances associated with particular IEMs

(a)  

3.

Enzyme missing in PKU

(a)  

4.

Screening test for PKU with gray to gray-green color as positive result

(a)  

5.

This inhibits the growth of the bacteria used in Guthrie Test

(a)  

6.

Result in Guthrie test with the ABSENCE of phenylalanine

(a)  

7.

Confirmatory test for PKU

(a)  

8.

Tyrosinemia/Tyrosiluria type if tyrosine aminotrasferase is missing/defecient

a)

Type 1

b)

Type 2

c)

Type 3

d)

Type 4

9.

Confirmatory test for Tyrosiluria (give one)

(a)  

10.

Rancid Butter Urine Disease

(a)  

11.

Hartnup Disease is also known as

(a)  

12.

Lesch-Nyhan Syndrome is characterized by an increase level of what substance in the urine?

(a)  

13.

Screening test for Alkaptonuria which gives a yellow precipitate

a)

FeCl3 Tube Test

b)

Clinitest

c)

Alkalinization of urine

14.

What is the product of the oxidation of 5,6-dihydroxyindole?

(a)  

15.

Urine turns black upon air exposure

a)

Alkaptonuria

b)

Melanuria

c)

Both

d)

Neither

16.

Which of the following is/are classified as Branched-Chain Amino Acid disorder?

a)

MSUD

b)

PKU

c)

Tyrosinuria

d)

Tyrosinemia

17.

Screening test for Indicanuria

(a)  

18.

Metabolite of Serotonin

(a)  

19.

Cystinosis is a defective tubular absorption of what amino acid/s?

a)

Ornithin

b)

Lysin

c)

Arginine

d)

Cystein

e)

None of the above

20.

Test for the presence of homocystine in urine/blood (no need to put the word "test")

(a)