Glycogen storage disorder,Fatty acid oxidation defects

4th Pediatric Infectious Diseases Conference

ISSN 0973 - 0958

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APPROACH TO HYPOGLYCEMIA

Related topics

Hypoglycemia : Clinical Features

Hypoglycemia : Major Causes

Hyperinsulinism and Ketotic Hypoglycemia

Fasting hypoglycemia and Erratic hypoglycemia

Treatment and Side effects of Hypoglycemia

Dr Ira Shah
M.D, DCH(Gold Medalist), FCPS, DNB

Glycogen storage disorder -

Glycogen synthetase deficiency (GSD Type 0) presents as early morning hypoglycemia. There is associated ketonemia but no hepatomegaly. Clinical picture is similar to ketotic hypoglycemia. Prolonged hyperglycemia after glucose administration with increase in serum lactate should indicate the deficiency. Hypoglycemia is unresponsive to glucagon administration as liver glycogen content is decreased.

Von Geirke's disease (GSD Type I) - The kidneys and liver glycogen content in increased and liver and kidneys are enlarged. Patients may have "doll's face", stunted growth, and normal mental development. Along with hypoglycemia, there is lactic acidosis, hyperlipidemia, and hyperuricemia. IV glucagon (post-prandial) does not raise the blood glucose. Continuous nighttime feeding by tube is required.

GSD Type III - Patients have hepatomegaly. However the kidneys are not enlarged (as in GSD type I). Serum concentrations of uric acid, lactate, ketones and lipids are normal. IV glucagon (postprandial) raises the blood glucose level.

Fatty acid oxidation defects -
Hypoglycemia is associated with hypoketonemia. Patients usually present in the 2^nd year of life and hypoglycemia is associated with encephalopathy mimicking Reye's syndrome. Diagnosis is established by evaluating plasma for organic acids and urine for dicarboxylic acid and enzyme studies from liver biopsy tissue or cultured fibroblasts.

Next : Fasting Hypoglycemia and Erratic Hypoglycemia

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