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Physiology section 3 > Glycogen Storage Diseases > Flashcards

Glycogen Storage Diseases Flashcards

1
Q

Name of type 1 GSD?

A

Von Gierke’s

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2
Q

Name of type 2 GSD?

A

Pompe’s

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3
Q

Name of type 3 GSD?

A

Cori’s

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4
Q

Name of type 4 GSD?

A

Anderson’s

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5
Q

Name of type 5 GSD?

A

McArdle’s

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6
Q

Name of type 6 GSD?

A

Her’s

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7
Q

How many types of GSDs exists?

A

8

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8
Q

What is the deficient enzyme in type 1?

A

Glucose-6-phosphatase

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9
Q

What enzyme is deficient in type 2?

A

1,4 Glucosidase

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10
Q

What enzyme is deficient in type 3?

A

Amylo - 1,6 - debranching

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11
Q

What enzyme is deficient in type 4?

A

Branching

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12
Q

What enzyme is deficient in type 5?

A

Phosphorylase

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13
Q

What enzyme is deficient in type 6?

A

Phosphorylase

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14
Q

What enzyme is deficient in type 7?

A

PFK-1

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15
Q

What enzyme is deficient in type 8?

A

Phosphorylase kinase

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16
Q

What organs are associated with type 1?

A

Liver and kidneys

17
Q

What organs are associated with type 2?

A

All organs

18
Q

What organs are associated with type 3?

A

Muscle and liver

19
Q

What organs are associated with type 4?

A

Liver and spleen

20
Q

What organs are associated with type 5?

21
Q

What organs are associated with type 6?

22
Q

What organs are associated with type 7?

23
Q

What organs are associated with type 8?

24
Q

Clinical conditions of type 1?

A
  • Enlarged liver
  • Failure to thrive
  • Gouty arthritis
  • severe hypoglycemia
  • Hyperlipidemia
  • Mental retardation
25
Clinical conditions of type 2?
- Cardiorespiratory failure - Death - Hypotonia cardiomegaly
26
Clinical conditions of type 3?
-milder type 1
27
Clinical conditions of type 4?
-Progressive cirrhosis of liver, death before age 2
28
Clinical conditions of type 5?
- Limited ability to perform strenuous activity | - produces muscle cramps
29
Clinical conditions of type 6?
milder type 1
30
Clinical conditions of type 7?
like type 5
31
Clinical conditions of type 8?
- liver enlargement | - hypoglycemia

Physiology section 3 (18 decks)

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