3) Explain the elevations noted for AST and ALT in the fructose tolerance test.

High liver enzymes mean that the liver is not functioning properly. In other words, it signifies that there
is some inflammation or damage in liver cells which is responsible for the release of excess amount of
certain chemicals like liver enzymes into the bloodstream. There are two particular liver enzymes that
get elevated in this condition. They are: Alanine Transaminase (ALT) and Aspartate Transaminase (AST).

Fibrosis and cirrhosis of the liver are some of the chronic results of hereditary fructose intolerance. In
the fructose tolerance test, the liver was in a state of induced damage caused by the intravenous
infusion of fructose. The liver would show histologic changes and would then release increased
amounts of ALT and AST enzymes.

Fructose taken orally is absorbed in the small intestine at about half the rate of glucose but disappears
from the blood twice the rate. The liver metabolizes most of this unless there is an excessive load such
as high doses given intravenously in which case some maybe converted by hexokinase to 6-phosphate
particularly in adipose or muscle tissues. Fructose has a high Vmax resulting at its phosphorylation which is
ten times higher than glucose also a decrease in ATP and Pi.

These other consequences follow: 1) the high concentration of fructose-1-phosphate inhibits

glycogenolysis exacerbated by low Pi, 2) the high concentration of ADP induces adenylate kinase to form
ATP and AMP with AMP being oxidatively degraded to uric acid and 3) the high concentration of
fructose-1-phosphate inhibits other important enzymes such as glucose-6-phosphate isomerase and
other residual aldolase which are essential for gluconeogenesis. Low ATP also contributes to this which
then explains the fructose-induced hypoglucosemia.

4) What are the symptoms of hypoglycemia not found in essential fructosuria (type 1)?

Essential fructosuria is caused by a deficiency of fructokinase which results in the slower removal of D-
fructose from the blood and fructose levels may stay elevated in the blood for several hours. This would
distinguish the occurrence of essential fructosuria from hypoglycemia.

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