Glycogen Storage Disease

Question 1

what is glycogen storage disease

Answer 1

An inherited disease characterized by the abnormal accumulation of glycogen in the tissues (esp. liver and kidneys)

Question 2

how many categories of glycogen storage disease?

Answer 2

6

Question 3

what is the most common glycogen storage disease type?

Answer 3

Type 1 or Von-Gierke’s disease

Question 4

what is Von Gierke’s disease?

Answer 4

Glycogen storage disease in which abnormally large amounts of glycogen are deposited in the liver and kidneys

Question 5

what is the sonographic appearance of glycogen storage disease

Answer 5

Hepatomegaly
increased echogenicity
slightly increased attenuation

Question 6

what does glycogen storage disease resemble

Answer 6

Fatty infiltration

Question 7

what is glycogen storage disease associated with?

Answer 7

Adenoma
focal nodular hyperplasia
hepatomegaly

Question 8

what does adenoma in glycogen storage disease present as

Answer 8

well-demarcated, round, homogenous, echogenic tumor

Question 9

what are differential diagnoses of glycogen storage disease

Answer 9

Focal nodular hyperplasia

fatty liver

Question 10

what is a clinical finding of glycogen storage disease?

Answer 10

Disturbance of acid-base balance