Glycogen Storage Diseases Flashcards by Joshua Beverly | Brainscape

Q

Name of type 1 GSD?

A

Von Gierke’s

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Q

Name of type 2 GSD?

A

Pompe’s

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Q

Name of type 3 GSD?

A

Cori’s

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Q

Name of type 4 GSD?

A

Anderson’s

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Q

Name of type 5 GSD?

A

McArdle’s

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Q

Name of type 6 GSD?

A

Her’s

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Q

How many types of GSDs exists?

A

8

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Q

What is the deficient enzyme in type 1?

A

Glucose-6-phosphatase

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Q

What enzyme is deficient in type 2?

A

1,4 Glucosidase

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Q

What enzyme is deficient in type 3?

A

Amylo - 1,6 - debranching

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Q

What enzyme is deficient in type 4?

A

Branching

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Q

What enzyme is deficient in type 5?

A

Phosphorylase

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Q

What enzyme is deficient in type 6?

A

Phosphorylase

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Q

What enzyme is deficient in type 7?

A

PFK-1

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Q

What enzyme is deficient in type 8?

A

Phosphorylase kinase

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Q

What organs are associated with type 1?

A

Liver and kidneys

Q

What organs are associated with type 2?

A

All organs

Q

What organs are associated with type 3?

A

Muscle and liver

Q

What organs are associated with type 4?

A

Liver and spleen

Q

What organs are associated with type 5?

A

Muscle

Q

What organs are associated with type 6?

A

Liver

Q

What organs are associated with type 7?

A

Muscle

Q

What organs are associated with type 8?

A

Liver

Q

Clinical conditions of type 1?

A

Enlarged liver
Failure to thrive
Gouty arthritis
severe hypoglycemia
Hyperlipidemia
Mental retardation

Q

Clinical conditions of type 2?

A

Cardiorespiratory failure
Death
Hypotonia cardiomegaly

Q

Clinical conditions of type 3?

A

-milder type 1

Q

Clinical conditions of type 4?

A

-Progressive cirrhosis of liver, death before age 2

Q

Clinical conditions of type 5?

A

Limited ability to perform strenuous activity

- produces muscle cramps

Q

Clinical conditions of type 6?

A

milder type 1

Q

Clinical conditions of type 7?

A

like type 5

Q

Clinical conditions of type 8?

A

liver enlargement

- hypoglycemia