Glycogen storage disease

GLYCOGEN STORAGE DISEASE

Glucose

  • Glucose is major source of energy. The body obtains glucose mainly from dietary carbohydrates.
  • Fructose obtained from the dietary sucrose and galactose obtained from dietary lactose are observed and converted into glucose in liver.
  • Most tissues also require glucose for the synthesis of ribose or glycoproteins.
  • Glucose and galactose are absorbed from the intestine readily than other monosaccarides.
  • During fasting blood glucose levels are maintained by both glycogenolysis and gluconeogenesis.
  • Several hormones, liver and extra hepatic tissues regulate the maintance of normal blood glucose .
  • The major disorders of glucose homeostasis are diabetes mellitus and the low blood glucose concentration.

Hypoglycemia

  • Due to B cell tumor, there is more production of insulin.
  • Insulin over doe
  • Glycogen storage disease – due to deficiency of glucose 6 phosphatase
  • Hepatic diseases.
  • Adrenocortical insufficiency
  • Sepsis- severe infection results in hypoglycemia.
  • Inadequate dietary intake of glucose and other substance required for hepatic gluconeogenesis.
  • Renal failure
  • Hypoglycemia in baby pigs occurs during the few days of life due to the insufficient gluconeogenic process.

Hyperglycemia

  • Insulin deficiency
  • Stress with an increase in glucocorticoids and catecholamine secretion.
  • Hyperadrenocorticism.
  • Pancreatic diseases.
  • Administration of general anesthetics.

Diabetes mellitus

  • Diabetes mellitus is the common endocrine disorder encounter in clinical practice. It is due to deficiency or resistance to insulin.
  • Based on the requirements of insulin, diabetes mellitus is generally sub classified as
    •  Insulin dependent diabetes mellitus
    •  Non insulin dependent diabetes mellitus

Glycogen storage diseases

It is type of genetis diseases, which reduce or prevent the release of glucose from glycogen, which lead to symptoms of hypoglycemia and excessive glycogen storage.

Name
Missing Enzyme
Symptoms
Von Gierke’s disease (type I) Glucose 6 phosphatase Enlarge liver & kidney, low blood sugar, slow growth
Pompe’s diseases (type II) lysosomal alpha-1,6,glucosidase Enlarge liver & heart, muscle weakness
Cori’s disease ( Type III) debranching enzyme phosphorylase Cirrhosis, muscle & heart damage
Type VIII Hepatic phosphorylase kinase b

Last modified: Monday, 4 June 2012, 6:28 AM