Fructose Disorder (Hereditary Fructose Intolerance)
Hereditary Fructose Intolerance (HFI) is a condition in which the ability of the affected individual to digest fructose is hampered. Fructose is present in fruits, sucrose (table sugar), and infant preparations containing sucrose.
Cause
HFI results due to the deficiency of fructose-1-phosphate aldolase enzyme, present in the liver,
Symptoms
HFI produces symptoms after fructose ingestion, thus they appear later than those of galactosemia. Failure to gain desired weight, vomiting, hypoglycaemia, liver dysfunction, and kidney defects are some symptoms of HFI. Liver and kidney gets damaged if foods containing fructose are ingested frequently. Secondary complications like hepatomegaly (enlargement of liver), and cirrhosis (chronic liver disease) may also occur due to liver damage. If ingestion of fructose is continued, seizures and coma may occur, and the individual ultimately dies due to liver and kidney failure. Since the symptoms experienced due to fructose ingestion are severe, most of the individuals suffering from hereditary fructose intolerance start disliking fruits, juices, and other fructose-containing foods.
Treatment
The treatment of HFI involves a strictly controlled diet of the patient.
All fructose-, sucrose-, and sorbitol-containing foods are strictly eliminated from their daily diet. Diet control eliminates the symptoms, which however relapses. on consuming fructose, sucrose, or sorbitol.
If the liver disease has reached a life- threatening stage, liver transplantation is recommended for treating HFI.
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