Storage diseases

Question 1

Another name for GSD I?

Answer 1

Von Geirkes disease

Question 2

Another name for GSD II?

Answer 2

Pompe’s Disease

Question 3

Another name for GSD III?

Answer 3

Cori’s Disease

Question 4

Another name for GSD IV?

Answer 4

Anderson’s Disease

Question 5

Another name for GSD V?

Answer 5

McArdle’s Disease

Question 6

Another name for GSD VI?

Answer 6

Hers’ Disease

Question 7

Another name for GSD VII?

Answer 7

VII

Question 8

Another name for GSD VIII?

Answer 8

VIII

Question 9

Defective enzyme in I?

Answer 9

G6 phosphotase

Question 10

Defective enzyme in II?

Answer 10

1-4 Glucosidase

Question 11

Defective enzyme in III?

Answer 11

Amylo 1-6 debranching

Question 12

Defective enzyme in IV?

Answer 12

Branching enzyme

Question 13

Defective enzyme in V?

Answer 13

Phosphorylase

Question 14

Defective enzyme in VI?

Answer 14

Phosphorylase

Question 15

Defective enzyme in VII?

Answer 15

PFKI

Question 16

Defective enzyme in VIII?

Answer 16

Phosphorulase Kinase

Question 17

Affected organ in I?

Answer 17

Liver & Kidney

Question 18

Affected organ in II?

Answer 18

All organs

Question 19

Affected organ in III?

Answer 19

Muscles & Liver

Question 20

Affected organ in IV?

Answer 20

Liver & Spleen

Question 21

Affected organ in V?

Answer 21

Muscle

Question 22

Affected organ in VI?

Answer 22

Liver

Question 23

Affected organ in VII?

Answer 23

Musle

Question 24

Affected organ in VIII?

Answer 24

Liver

Question 25

Which diseases only affect the liver?

Answer 25

VIII & VI

Question 26

Which diseases only affect muscle?

Answer 26

V & VII

Question 27

What happens to glycogen in GSD I?

Answer 27

Increased amount, normal sturcture

Question 28

What happens to glycogen in GSD II?

Answer 28

Massive Increase in amount, normal structure

Question 29

What happens to glycogen in GSD III?

Answer 29

Increased amount, short outer branches

Question 30

What happens to glycogen in GSD IV?

Answer 30

Normal amount, Long outer branches

Question 31

What happens to glycogen in GSD V?

Answer 31

Moderate increase in amount, normal sturcture

Question 32

What happens to glycogen in GSD VI?

Answer 32

Increased amount, normal structure

Question 33

What happens to glycogen in GSD VII?

Answer 33

Increased Amount, normal structure

Question 34

What happens to glycogen in GSD VIII?

Answer 34

Increased Amount, normal structure

Question 35

Which GSDs demonstrate normal structure?

Answer 35

1, 2, 5-8

Question 36

Which GSDs have average increase in amount?

Answer 36

1, 3, 6-8

Question 37

Which GSD has longer outer branches?

Answer 37

IV

Question 38

Which GSD has short outer branches?

Answer 38

III

Question 39

Symptoms of GSD I?

Answer 39

- Hepatomeglia - Failure to thrive - Sever hypoglycemia - Ketosis - Hyperlipemia - Hyperuricemia - High fasting level serum fatty acids

Question 40

Symptoms of GSD II?

Answer 40

- Cardiorespiratory failure | - Death before 2 yo

Question 41

Symptoms of GSD III?

Answer 41

Same as type one but milder

Question 42

Symptoms of GSD IV?

Answer 42

- Progressive cirrohosis of liver | - Liver failure usually causes death before age two?

Question 43

Symptoms of GSD V?

Answer 43

- Limited ability for strenuous exercise due to painful cramps - Otherwise normal and well developed

Question 44

Symptoms of GSD VI?

Answer 44

Type I but milder

Question 45

Symptoms of GSD VII?

Answer 45

Like type V

Question 46

Symptoms of GSD VIII?

Answer 46

- Mild liver enlargement | - Mild Hypoglycemia

Question 47

Which GSDs have similiar symptoms?

Answer 47

- 1, 3, 6: 3 & 6 milder | - 5 & 7

Question 48

What GDD has following symptoms: - Mild liver enlargement - Mild Hypoglycemia

Answer 48

VIII

Question 49

What GDD has following symptoms: - Limited ability for strenuous exercise due to painful cramps - Otherwise normal and well developed

Answer 49

VII & V

Question 50

What GDD has following symptoms:

Answer 50

VI

Question 51

What GDD has following symptoms: - Hepatomeglia - Failure to thrive - Sever hypoglycemia - Ketosis - Hyperlipemia - Hyperuricemia - High fasting level serum fatty acids

Answer 51

I, III, VI

Question 52

What GDD has following symptoms: - Limited ability for strenuous exercise due to painful cramps - Otherwise normal and well developed

Answer 52

V

Question 53

What GDD has following symptoms: - Progressive cirrohosis of liver - Liver failure usually causes death before age two?

Answer 53

IV

Question 54

What GDD has following symptoms: - Cardiorespiratory failure - Death before 2 yo

Answer 54

II

Question 55

Name acronym?

Answer 55

``` Veinte Purple Cats Ate My Ham ```