38 Questions
What is the primary function of the small intestine in glucose metabolism?
Mediating the uptake of glucose into cells
What is the result of glycogen storage diseases affecting the liver?
Hepatomegaly and hypoglycemia
What is the purpose of continuous drip feedings in GSD type I patients?
To supply continuous glucose
What is the treatment for the muscular forms of glycogen storage diseases?
Avoidance of strenuous exercise
Which organs rely on a constant supply of glucose?
Brain, red blood cells, and adrenal medulla
What is the purpose of uncooked cornstarch in GSD type I treatment?
To provide slow-release glucose
What is the result of defects in earlier steps of glycogen breakdown in the liver?
Milder versions of GSD type I disease
What is the last step in glucose release from the liver affected in GSD type I?
Glucose release
What is the primary source of energy for the body?
Carbohydrates
What is the storage form of carbohydrates in humans?
Glycogen
What happens when carbohydrates are not broken down properly in the body?
A harmful amount of sugar builds up in the body
What is the name of the hormone that helps transport glucose into cells?
Insulin
What is the term for high blood sugar levels?
Hyperglycemia
What is the leading cause of adult blindness?
Diabetes
What is the term for low blood sugar levels?
Hypoglycemia
How are most carbohydrate metabolism disorders treated?
With specific dietary interventions
What is a common long-term complication of diabetes?
Kidney failure
At what age does type 1 diabetes often begin?
Late childhood
What is the primary cause of type 1 diabetes?
Autoimmune disorder
How is type 1 diabetes managed?
Injections of insulin
What is the primary cause of type 2 diabetes?
Insulin resistance
What is the effect of excess body fat on type 2 diabetes?
Increases risk
What is the primary goal of lifestyle intervention in type 2 diabetes?
To delay or prevent the condition
What organs are most commonly affected by glycogen storage diseases?
Liver and skeletal muscle
What is the process by which glucose is manufactured from amino acids and pyruvate?
Gluconeogenesis
What is the main consequence of a reduced rate of glycolysis in erythrocytes?
Nonspherocytic hemolytic anaemia
What is the primary source of energy for erythrocytes?
Glycolysis
What is the consequence of galactosemia if left untreated or if treatment is delayed?
Intellectual disability
What is the primary step affected in pyruvate kinase deficiency?
Step 10 of glycolysis
What is the result of galactose-1-phosphate accumulation in galactosemia?
Clinical manifestations of galactosemia
What is the consequence of a galactose-rich diet in galactosemia?
Cataracts
What is the vulnerable group to severe bacterial infections, especially by Escherichia coli, in galactosemia?
Infants
What is the primary treatment for galactosemia?
Exclusion of galactose from the diet
What is the role of fructose-1-phosphate aldolase in the body?
To convert fructose-1-phosphate to fructose-1,6-diphosphate
What is a common symptom of hereditary fructose intolerance (HFI)?
Failure to gain weight satisfactorily
What is the mainstay of therapy for fructose 1,6-diphosphatase deficiency?
Rapid treatment of hypoglycemic episodes with intravenous fluids containing glucose
Why do children with hereditary fructose intolerance (HFI) tend to avoid sweet foods?
Because sweet foods make them sick
What is a characteristic of the teeth of children with hereditary fructose intolerance (HFI)?
They are notable for the absence of caries
Learn about glycogen storage diseases that affect the liver and skeletal muscle, and how they impact glucose metabolism in the body.
Make Your Own Quizzes and Flashcards
Convert your notes into interactive study material.
Get started for free
