DEFINITION
Carbohydrates are sugars. There are simple sugars and
complex sugars. Cane sugar (sucrose) is composed of two simple sugars, glucose
and fructose. Lactose (milk sugar) is composed of glucose and galactose. Both
sucrose and lactose must be solved beforehand into its component sugars by
enzymes before it can be absorbed and used by the body.
Carbohydrates are found in bread, pasta, rice, and other
foods containing carbohydrates are long chains of molecules of simple sugars.
These long molecules must also be solved by the body. If the enzymes needed to
process a certain sugar is not there, then the sugar can accumulate in the body
and cause interference.
There are several disorders related to the metabolism of
carbohydrates:
Glycogen Accumulation disease (Glycogen Storage Disease).
The disease is caused by abnormalities of enzymes involved in glycogen
metabolism, causing a variety of disorders, such as stunted growth, weakness,
and confusion.
Galactosemia. Galactosemia is a condition in which the
levels of galactose in the blood is high. This disorder is caused by a lack of
one of the enzymes that are essential for the metabolism of galactose, the
sugar found in lactose. Metabolites that are toxic to the liver and kidneys
accumulate. This metabolite also damages the eye's lens, causing cataracts.
Hereditary Fructose Intolerance. This disorder is caused by
a lack of enzymes required for the metabolism of fructose. Fructose is the
sugar found in sugar cane and fruits. As a result, a byproduct of fructose
accumulate in the body, inhibiting the formation of glycogen and the conversion
of glucose to produce energy. Consuming fructose is more than small amounts
lead to low blood sugar levels and can cause kidney and liver damage.
Mukopolisakaridosis. Mukopolisakaridosis is a group of
inherited disorders in which the complex sugar molecules can not be solved and
accumulate in amounts harmful to the body tissues. This is caused by a lack of
enzymes needed to break and store complex sugar molecules (mucopolysaccharides).
The result is a variety of abnormalities in the bones, eyes, liver, and spleen,
sometimes accompanied by intellectual impairment.
Pyruvate metabolism disorders. Pyruvate is a substance that
is formed in the processing of carbohydrates and protein, which serves as a
source of energy for the body's cells. Pyruvate metabolism disorders can limit
the ability of cells to produce energy and leads to accumulation of lactic
acid.
REFERENCE
- Lee M Sanders. Disorders of Carbohydrate Metabolism. Merck
Manual. 2009.
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