SUBJECTS
|
BROWSE
|
CAREER CENTER
|
POPULAR
|
JOIN
|
LOGIN
Business Skills
|
Soft Skills
|
Basic Literacy
|
Certifications
About
|
Help
|
Privacy
|
Terms
|
Email
Search
Test your basic knowledge |
Lysosomal Storage Diseases
Start Test
Study First
Subject
:
health-sciences
Instructions:
Answer 22 questions in 15 minutes.
If you are not ready to take this test, you can
study here
.
Match each statement with the correct term.
Don't refresh. All questions and answers are randomly picked and ordered every time you load a test.
This is a study tool. The 3 wrong answers for each question are randomly chosen from answers to other questions. So, you might find at times the answers obvious, but you will see it re-enforces your understanding as you take the test each time.
1. Accumulates in Gaucher's disease
Galactosylceramidase (= absence of galactosylceramide & galactoside)
Iduronate sulfatase
Glucocerebroside in brain - liver - spleen - and bone marrow
Hepatosplenomegaly - anemia - thrombocytopenia - 'crinkled paper' cells
2. Accumulates in Niemann - Pick disease
Glucocerebroside in brain - liver - spleen - and bone marrow
Sphingomyelin cholesterol in reticuloendothelial & parenchymal cells & tissues
Dead by age 3
GM2 ganglioside
3. Accumulates in Krabbe's disease
Sphingomyelinase
Normal until 3-6 months of age - then weakness begins - development slows & regresses - exaggerated startle response - death by 3
Galactocerebroside in brain
Galactosylceramidase (= absence of galactosylceramide & galactoside)
4. Deficient in metachromatic leukodystrophy
Hepatosplenomegaly - anemia - thrombocytopenia - 'crinkled paper' cells
Optic atrophy - spasticity - early death
Galactosylceramidase (= absence of galactosylceramide & galactoside)
Arylsulfatase A
5. Accumulates in Fabry's disease
Sphingomyelin cholesterol in reticuloendothelial & parenchymal cells & tissues
Alpha - L- iduronidase
Mild mental retardation - no corneal clouding
Ceramide trihexoside in heart - brain - and kidneys
6. Findings in Hurler's syndrome
Fabry's disease Hunter's disease
Corneal clouding & mental retardation
Ceramide trihexoside in heart - brain - and kidneys
Renal failure - increased risk of stroke & MI
7. Findings in Fabry's disease
Fabry's disease Hunter's disease
Renal failure - increased risk of stroke & MI
Ceramide trihexoside in heart - brain - and kidneys
Optic atrophy - spasticity - early death
8. Accumulates in Tay- Sachs disease
GM2 ganglioside
Mild mental retardation - no corneal clouding
Fabry's disease Hunter's disease
Optic atrophy - spasticity - early death
9. Findings in Krabbe's disease
Ceramide trihexoside in heart - brain - and kidneys
Normal until 3-6 months of age - then weakness begins - development slows & regresses - exaggerated startle response - death by 3
Optic atrophy - spasticity - early death
Mild mental retardation - no corneal clouding
10. Deficient in Krabbe's disease
Sulfatide in the brain - kidney - liver - and peripheral nerves
Galactocerebroside in brain
Hexosaminadase
Galactosylceramidase (= absence of galactosylceramide & galactoside)
11. Deficient in Hurler's syndrome
Sphingomyelin cholesterol in reticuloendothelial & parenchymal cells & tissues
Alpha - L- iduronidase
Optic atrophy - spasticity - early death
Fabry's disease Hunter's disease
12. Deficient in Gaucher's disease
Normal until 3-6 months of age - then weakness begins - development slows & regresses - exaggerated startle response - death by 3
Sphingomyelin cholesterol in reticuloendothelial & parenchymal cells & tissues
GM2 ganglioside
Glucocerebrosidase
13. Deficient in Hunter's syndrome
Hepatosplenomegaly - anemia - thrombocytopenia - 'crinkled paper' cells
Mild mental retardation - no corneal clouding
Fabry's disease Hunter's disease
Iduronate sulfatase
14. Findings in Niemann - Pick disease type A
Galactosylceramidase (= absence of galactosylceramide & galactoside)
GM2 ganglioside
Sulfatide in the brain - kidney - liver - and peripheral nerves
Dead by age 3
15. Deficient in Niemann - Pick disease
GM2 ganglioside
Sphingomyelinase
Corneal clouding & mental retardation
Alpha - galactosidase A
16. Deficient in Tay- Sachs disease
Optic atrophy - spasticity - early death
Galactosylceramidase (= absence of galactosylceramide & galactoside)
Alpha - galactosidase A
Hexosaminadase
17. Findings in Hunter's syndrome
Galactocerebroside in brain
Dead by age 3
Corneal clouding & mental retardation
Mild mental retardation - no corneal clouding
18. Findings in Tay- Sachs disease
Alpha - L- iduronidase
Normal until 3-6 months of age - then weakness begins - development slows & regresses - exaggerated startle response - death by 3
Galactocerebroside in brain
Iduronate sulfatase
19. Deficient in Fabry's disease
Ceramide trihexoside in heart - brain - and kidneys
Alpha - galactosidase A
GM2 ganglioside
Corneal clouding & mental retardation
20. X- linked recessive Lysosomal Storage Diseases
Warning
: Invalid argument supplied for foreach() in
/var/www/html/basicversity.com/show_quiz.php
on line
183
21. Accumulates in metachromatic leukodystrophy
Galactosylceramidase (= absence of galactosylceramide & galactoside)
Normal until 3-6 months of age - then weakness begins - development slows & regresses - exaggerated startle response - death by 3
Sulfatide in the brain - kidney - liver - and peripheral nerves
Fabry's disease Hunter's disease
22. Findings in Gaucher's disease
Warning
: Invalid argument supplied for foreach() in
/var/www/html/basicversity.com/show_quiz.php
on line
183