Lsds

Front 1

MPS 1

Back 1

Hurler Syndrome

Front 2

MPS II

Back 2

Hunter Syndrome

Front 3

MPS III

Back 3

Sanfilippo Syndrome

Front 4

MPS IV

Back 4

Morquio Syndrome

Front 5

Substrate accumulated from mucopolysaccharidoses (MPS)

Back 5

Glycosaminoglycans (GAGs)

Front 6

What are the diseases caused by mucopolysaccharidoses (MPS)

Back 6

1. Hurlers

2. Hunters

3. Sanfilippo

4. Morquio

Front 7

Hurlers Syndrome has deficiency in which enzyme?

Back 7

alpha-L-iduronidase

Front 8

Hunters Syndrome has deficiency in which enzyme?

Back 8

Iduronate-2-sulfatase

Front 9

MPS I accumulates which GAGs?

Back 9

Dermatan Sulfate

Heparan Sulfate

Front 10

MPS II accumulates which GAGs?

Back 10

Dermatan Sulfate

Heparan Sulfate

Front 11

MPS III accumulates which GAGs?

Back 11

Heparan Sulfate

Front 12

MPS IV accumulates which GAGs?

Back 12

Keratan Sulfate

Front 13

Which LSD exhibits claw hands?

Back 13

Hurlers and Hunters

Front 14

Which LSD exhibits corneal clouding?

Back 14

Hurlers

Front 15

Which LSD exhibits non-corneal clouding?

Back 15

Hunters

Front 16

Which LSD exhibits spondylo epiphyseal dysplasia and genu valgum?

Back 16

Morquio

Front 17

Substrates accumulated from sphingolipidoses?

Back 17

Sphingolipids

Front 18

Sandhoff's Disease accumulates which sphingolipid?

Back 18

Gangliosides (GM2) and globosides

Front 19

Tay-Sach's Disease accumulates which sphingolipid?

Back 19

Gangliosides (GM2)

Front 20

Niemann-Pick accumulates which sphingolipid?

Back 20

Sphingomylein

Front 21

Gauchers Disease accumulates which sphingolipid?

Back 21

Glucosylceramide

Front 22

Fabry Disease accumulates which sphingolipid?

Back 22

Globosides

Front 23

Fabers Disease accumulates which sphingolipid?

Back 23

Ceramide

Front 24

Metachromatic Leukodystrophy Disease accumulates which sphingolipid?

Back 24

Cerebroside sulfate

Front 25

Krabbe's Disease accumulates which sphingolipid?

Back 25

Galactocerebrosidaside

Front 26

Sandhoff's Disease is deficient in which enzyme?

Back 26

Beta-Hexoaminidase A & B

Front 27

Tay-Sach's Disease is deficient in which enzyme​?

Back 27

Beta-Hexoaminidase A

Front 28

Niemann-Pick is deficient in which enzyme​?

Back 28

Sphingomyelinase

Front 29

Gaucher's Disease is deficient in which enzyme​?

Back 29

Beta-Glucocerebrosidase

Front 30

Fabry Disease is deficient in which enzyme​?

Back 30

Alpha-Galactosidase A

Front 31

Faber's Disease is deficient in which enzyme​?

Back 31

Ceramidase

Front 32

Metachromatic Leukodystrophy Disease is deficient in which enzyme​?

Back 32

Arylsulfatase A

Front 33

Krabbe's Disease is deficient in which enzyme​?

Back 33

Galactocerebrosidase

Front 34

Which LSDs have cherry-red maculae?

Back 34

Sandhoff's

Tay-Sachs

Niemann-Pick

Front 35

Which LSDs are common amongst Ashkenazi Jew?

Back 35

Tay-Sachs

Niemann-Pick

Gaucher's

Front 36

Which LSDs have onion skin (zebra bodies)?

Back 36

Tay-Sachs

Niemann-Pick

Front 37

How does Tay-Sachs differ from Sandhoff's Disease?

Back 37

Sandhoff's exhibits organomegaly

Front 38

How does Tay-Sachs and Sanhoff's differ from Niemann-Pick Disease?

Back 38

Sandhoff's and Tay-Sachs exhibits seizures

Front 39

Which LSD has Erlenmeyer flask deformity?

Back 39

Gauchers

Front 40

Which LSD has "wrinkled paper" macrophages?

Back 40

Gauchers

Front 41

Which LSD has angiokeratomas?

Back 41

Fabry

Front 42

Which LSD affects PNS but not CNS?

Back 42

Fabry

Front 43

Which LSD cuases similarities to rheumetoid arthritis?

Back 43

Faber's

Front 44

Which LSD affects both PNS and CNS?

Back 44

Metachromatic Leukodystrophy

Front 45

Which LSD has gobloid cells?

Back 45

Krabbe's

Front 46

Which LSDs are X-linked?

Back 46

Hunter and Fabry

Front 47

What is the inheritance pattern of most LSDs?

Back 47

Autosomal Recessive

Front 48

What is the most common sphingolipidoses LSD?

Back 48

Gauchers

Front 49

What is the most common MPS LSD?

Back 49

Sanfilippo

Front 50

Which LSD is common to French Canadians?

Back 50

Morquio