CARBOHYDRATE

WHAT ARE CARBOHYDRATE?

Carbohydrate are organic compound that consists of carbon, hydrogen and oxygen in a 1:2:1 ratio. Sugars and other carbohydrates are the most abundance of all biological molecules on the planet. In cells, different kinds are stored or put to use as structural materials and as sources of instant energy.

Three main kinds of carbohydrate are i. Monosaccharide ii. Disaccharides iii. polysaccharides

MONOSACCHARIDES
One sugar unit Glucose

DISACCHARIDES
Two combined monosaccharide molecules. Examples: i. Fructose ii. Sucrose iii. Galactose

POLYSACCHARIDES
Chains of many monosaccharide molecules. Examples i. Cellulose ii. Chitin iii. starch iv. Glycogen

CARBOHYDRATE METABOLISMS
Glycolysis Glycogenesis Gluconeogenesis Glucogenolysis Pentose phosphate pathways

WHAT WILL HAPPED IF THERE ARE PROBLEMS IN THE CARBOHYDRATE METABOLISMS? IT WILL CAUSE SEVERAL CARBOHYDRATE METABOLISM DISORDER.

ONE OF THE CARBOHYDRATE METABOLISM DISORDERS IS FRUCTOSEMIA

Fructosemia?
Fructosemia is also called as: Heredity fructose intolerance (HFI) Fructose poisoning Fructose-1-phosphate aldolase deficiency Heredity fructosemia

What is HFI?
Is an autosomal recessive disorder thus there are no abnormal findings This disorder is inherited Individual who are obligate homozygous recessive of HFI will affected by several factor due to the carbohydrate metablolism disorder.

What is the cause of HFI?

unable to digest fructose (fruit sugar) or its precursors (sugar, sorbitol and brown sugar) unable to metabolise the fructose (especially in fruits and vegetables) for metabolic activities due to deficiency of activity of the enzyme fructose-1-phosphate aldolase (aldolase B)

resulting in the accumulation of fructose-1phosphate Aldolase B is the major aldolase isozyme in the liver and functions in both fructose metabolism and in gluconeogenesis Fructose-1-phosphate will not be converted to DHAP (dihydroacetone phosphate) and glyceraldehydes.

besides, it will traps phosphate that are needed for phosphorylation by glycogen phosphorylase that functioning to release glucose-1-phosphate from glycogen in the process of glucogenolysis. The glucose-1-phosphate should be converted to glucose-6-phosphate and then dephosphorylated to form glucose

Symptoms of HFI
Peoples with this disorder: usually develop a strong dislike for sweets and fruit. (especially newborn or weaning infants) Fructose presence in blood and urine. Abdominal pain Vomiting

Hypoglycaemia Jaundice Hyperuricemia Kidney failure Enlarged liver especially among infants Good dental hygiene among children Retarded growth

Treatment
Avoid taking foods and drinks with the fructose contents.

Effects of untreated HFI

Early diagnosis can help to normal lives if they adopt a fructose-free diet. If untreated, it can lead to: i. Serious liver (hepatic failure) and kidney damages. ii. Organ shock or coma due to hypoglycaemia and acidosis. iii. Hard pain and Mortality