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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. Deficient in Gaucher's disease
Iduronate sulfatase
Hexosaminadase
Glucocerebrosidase
Galactocerebroside in brain
2. Deficient in Krabbe's disease
Alpha - L- iduronidase
Galactosylceramidase (= absence of galactosylceramide & galactoside)
Normal until 3-6 months of age - then weakness begins - development slows & regresses - exaggerated startle response - death by 3
Glucocerebroside in brain - liver - spleen - and bone marrow
3. Findings in Gaucher's disease
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4. Deficient in Niemann - Pick disease
Fabry's disease Hunter's disease
Dead by age 3
Sphingomyelinase
Glucocerebrosidase
5. Accumulates in Gaucher's disease
Sphingomyelinase
Dead by age 3
Glucocerebroside in brain - liver - spleen - and bone marrow
Galactocerebroside in brain
6. Findings in Niemann - Pick disease type A
Alpha - galactosidase A
Dead by age 3
Sphingomyelinase
Optic atrophy - spasticity - early death
7. Accumulates in Krabbe's disease
Galactosylceramidase (= absence of galactosylceramide & galactoside)
Galactocerebroside in brain
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
8. Accumulates in Niemann - Pick disease
Hepatosplenomegaly - anemia - thrombocytopenia - 'crinkled paper' cells
Sphingomyelin cholesterol in reticuloendothelial & parenchymal cells & tissues
Ceramide trihexoside in heart - brain - and kidneys
Mild mental retardation - no corneal clouding
9. Findings in Hurler's syndrome
Alpha - galactosidase A
Glucocerebrosidase
Corneal clouding & mental retardation
Galactocerebroside in brain
10. Deficient in Fabry's disease
Arylsulfatase A
Alpha - galactosidase A
Sulfatide in the brain - kidney - liver - and peripheral nerves
Sphingomyelin cholesterol in reticuloendothelial & parenchymal cells & tissues
11. Deficient in Tay- Sachs disease
Ceramide trihexoside in heart - brain - and kidneys
Dead by age 3
Hexosaminadase
Alpha - L- iduronidase
12. Findings in Krabbe's disease
Alpha - galactosidase A
Optic atrophy - spasticity - early death
Renal failure - increased risk of stroke & MI
Arylsulfatase A
13. Findings in Fabry's disease
Glucocerebrosidase
Sphingomyelin cholesterol in reticuloendothelial & parenchymal cells & tissues
Mild mental retardation - no corneal clouding
Renal failure - increased risk of stroke & MI
14. Findings in Hunter's syndrome
GM2 ganglioside
Sulfatide in the brain - kidney - liver - and peripheral nerves
Mild mental retardation - no corneal clouding
Alpha - galactosidase A
15. X- linked recessive Lysosomal Storage Diseases
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16. Accumulates in Tay- Sachs disease
Iduronate sulfatase
Hepatosplenomegaly - anemia - thrombocytopenia - 'crinkled paper' cells
Alpha - galactosidase A
GM2 ganglioside
17. Deficient in metachromatic leukodystrophy
Hepatosplenomegaly - anemia - thrombocytopenia - 'crinkled paper' cells
Fabry's disease Hunter's disease
Arylsulfatase A
Optic atrophy - spasticity - early death
18. Accumulates in metachromatic leukodystrophy
Sulfatide in the brain - kidney - liver - and peripheral nerves
Fabry's disease Hunter's disease
Galactosylceramidase (= absence of galactosylceramide & galactoside)
Renal failure - increased risk of stroke & MI
19. Deficient in Hunter's syndrome
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
Iduronate sulfatase
Dead by age 3
20. Accumulates in Fabry's disease
Fabry's disease Hunter's disease
Galactosylceramidase (= absence of galactosylceramide & galactoside)
Ceramide trihexoside in heart - brain - and kidneys
Renal failure - increased risk of stroke & MI
21. Findings in Tay- Sachs disease
Alpha - galactosidase A
Corneal clouding & mental retardation
Mild mental retardation - no corneal clouding
Normal until 3-6 months of age - then weakness begins - development slows & regresses - exaggerated startle response - death by 3
22. Deficient in Hurler's syndrome
Alpha - L- iduronidase
Hexosaminadase
Optic atrophy - spasticity - early death
Corneal clouding & mental retardation