Glycogen Storage Diseases and Glucose Metabolism

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What is the primary function of the small intestine in glucose metabolism?

Releasing glucose into the bloodstream

Storing glycogen in the liver

Mediating the uptake of glucose into cells (correct)

Breaking down glycogen into glucose

What is the result of glycogen storage diseases affecting the liver?

Exercise intolerance and progressive weakness

Adrenal gland dysfunction

Brain damage and red blood cell deficiency

Hepatomegaly and hypoglycemia (correct)

What is the purpose of continuous drip feedings in GSD type I patients?

To promote liver function

To reduce hypoglycemia

To supply continuous glucose (correct)

To increase muscle strength

What is the treatment for the muscular forms of glycogen storage diseases?

Avoidance of strenuous exercise Signup and view all the answers

Which organs rely on a constant supply of glucose?

Brain, red blood cells, and adrenal medulla Signup and view all the answers

What is the purpose of uncooked cornstarch in GSD type I treatment?

To provide slow-release glucose Signup and view all the answers

What is the result of defects in earlier steps of glycogen breakdown in the liver?

Milder versions of GSD type I disease Signup and view all the answers

What is the last step in glucose release from the liver affected in GSD type I?

Glucose release Signup and view all the answers

What is the primary source of energy for the body?

Carbohydrates Signup and view all the answers

What is the storage form of carbohydrates in humans?

Glycogen Signup and view all the answers

What happens when carbohydrates are not broken down properly in the body?

A harmful amount of sugar builds up in the body Signup and view all the answers

What is the name of the hormone that helps transport glucose into cells?

Insulin Signup and view all the answers

What is the term for high blood sugar levels?

Hyperglycemia Signup and view all the answers

What is the leading cause of adult blindness?

Diabetes Signup and view all the answers

What is the term for low blood sugar levels?

Hypoglycemia Signup and view all the answers

How are most carbohydrate metabolism disorders treated?

With specific dietary interventions Signup and view all the answers

What is a common long-term complication of diabetes?

Kidney failure Signup and view all the answers

At what age does type 1 diabetes often begin?

Late childhood Signup and view all the answers

What is the primary cause of type 1 diabetes?

Autoimmune disorder Signup and view all the answers

How is type 1 diabetes managed?

Injections of insulin Signup and view all the answers

What is the primary cause of type 2 diabetes?

Insulin resistance Signup and view all the answers

What is the effect of excess body fat on type 2 diabetes?

Increases risk Signup and view all the answers

What is the primary goal of lifestyle intervention in type 2 diabetes?

To delay or prevent the condition Signup and view all the answers

What organs are most commonly affected by glycogen storage diseases?

Liver and skeletal muscle Signup and view all the answers

What is the process by which glucose is manufactured from amino acids and pyruvate?

Gluconeogenesis Signup and view all the answers

What is the main consequence of a reduced rate of glycolysis in erythrocytes?

Nonspherocytic hemolytic anaemia Signup and view all the answers

What is the primary source of energy for erythrocytes?

Glycolysis Signup and view all the answers

What is the consequence of galactosemia if left untreated or if treatment is delayed?

Intellectual disability Signup and view all the answers

What is the primary step affected in pyruvate kinase deficiency?

Step 10 of glycolysis Signup and view all the answers

What is the result of galactose-1-phosphate accumulation in galactosemia?

Clinical manifestations of galactosemia Signup and view all the answers

What is the consequence of a galactose-rich diet in galactosemia?

Cataracts Signup and view all the answers

What is the vulnerable group to severe bacterial infections, especially by Escherichia coli, in galactosemia?

Infants Signup and view all the answers

What is the primary treatment for galactosemia?

Exclusion of galactose from the diet Signup and view all the answers

What is the role of fructose-1-phosphate aldolase in the body?

To convert fructose-1-phosphate to fructose-1,6-diphosphate Signup and view all the answers

What is a common symptom of hereditary fructose intolerance (HFI)?

Failure to gain weight satisfactorily Signup and view all the answers

What is the mainstay of therapy for fructose 1,6-diphosphatase deficiency?

Rapid treatment of hypoglycemic episodes with intravenous fluids containing glucose Signup and view all the answers

Why do children with hereditary fructose intolerance (HFI) tend to avoid sweet foods?

Because sweet foods make them sick Signup and view all the answers

What is a characteristic of the teeth of children with hereditary fructose intolerance (HFI)?

They are notable for the absence of caries Signup and view all the answers