Glycogen Storage Diseases - Medicinequizzes.com

Welcome to your Glycogen / Storage diseases

Which of the statements is not correct?

There are two types of bonds found in glycogen molecules, alpha-1,4-glycosidic bonds and alpha-1,6-glycosidic bonds

Glycogen is mostly stored in liver and muscle tissue

Glycogen stored in muscle tissue mainly contributes to the blood glucose level

The branches of glycogen molecules are made up of Alpha-1,6-glycosidic bonds

All of them are correct

Which of the statements is not correct?

Epinephrine induces Glycogen synthesis

Phosphorylation of either enzymes, Glycogen synthase and Glycogen phosphorylase favours Glycogen synthesis

Vitamin B6 is necessary for Glycogen phosphorylase stabilization

Limit dextrins are 2 to 4 bond glucose molecules starting with the first glucose molecule of a branch of a glycogen molecule that can not be cleaves by Glycogen phosphorylase but Debranching enzyme

All of them are correct

Which of the statements is not correct?

In the first hours of fasting the primary source of glucose is from the previously ingested meals

After that Glycogen break delivers the Glucose needed

With time fatty acids and proteins start to gain of role as a source of gluconeogenesis

All of them are correct

None of them are correct

Which statements regarding Von Gierke’s disease is not correct?

It is also known as Glycogen storage disease type 1

Either deficiency of the enzyme Glycogen 6 phosphatase or a defect in the enzymes transport are known to cause this condition

Since muscles can use Glucose 6 phosphate for glycolysis, muscle weakness is less severe

The diet should mainly consist of disaccharides like fructose and sucrose to prevent hypoglycaemia due to deficient glycolysis in the liver

All statements are true

A 2-year-old male child is brought to the pediatric clinic with complaints of heavy breathing and pale skin. The parents say that the symptoms have been existing for the last two weeks and mostly start to occur 3 – 4 hrs after a meal. On physical examination, the physician noticed a distended abdomen. Low blood glucose was measured. The physician suspects a glycogen storage disease. Which of the following statements about the children’s condition would not be correct?

A phosphatase enzyme deficiency may be deficient

Subtype 1b is a deficiency in a glucose transporter

Lactic acidosis can be seen during fasting

Hepatomegaly occurs due to glycogen accumulation within the liver

Seizures are known to occur during eating due to a sudden increase in blood sugar

Which statement about Glycogen storage disease type three is not correct?

There is a deficiency in the debranching enzyme

Hypoglycemia is less severe due to intact gluconeogenesis of the liver

Muscle weakness is one of the main symptoms

Glycogen accumulation is seen in the muscles

Lactic acidosis is characteristic

Which statement about Glycogen storage disease type five is not correct?

A deficiency in the enzyme glycogen phosphorylase in muscle tissues is the cause

Hypoglycemia is a commonly seen

There is a problem in the breakdown of glycogen to glucose-1-phosphate in muscle tissues

Muslce weakness is seen

Myoglobulinuria results after excersize due to muscle damage

Which is not correct about Pompe disease

Deficiency in lysosomal a 1,4 glucosidase is the reason

Heart hypertrophy can be seen

The late-onset subtype is the severe type and results in death in infancy and childhood

Accumulation of glycogen in lysosomes is seen

All of them are correct

A 1-month-old newborn was brought for routinely examination. On physical examination the child showed a weak muscle tone and signs for respiratory distress. During oral examination the large tongue caught the attention of the examiner. The mother told that they have feeding problems and describes her child to be floppy when lifting her. The diagnosis was positive for a glycogen storage disease (GSD). Which statement regarding the specific type of GSDs is not correct?

A deficiency of lysosomal alpha 1,4 glucosidase is cause here

Because cells can not utilize glucose properly, muscle wasting is seen resulting in the characteristic Hypotonia of this condition

Cardiomegaly is specifically seen in the early onset of this disease

Heart failure due to this condition can cause hepatomegaly

All oft hem are correct

Which is not correct about Glycogen storage diseases?

Even though a defect in Glycogenolysis is seen in their different types, symptoms vary between them

Hypoglycemia is not typically seen in Cori and Pompe’s diseases due to functioning Gluconeogenesis in liver cells

People with Mc Ardle’s disease can present with dark urine cause by myoglobin because of muscle cell breakdown

Leading cause for death in Pompe’s disease is cardiomegaly

All of them are correct

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