What is HFI?
Scientific Definition: HFI is an autosomal recessive disorder of fructose metabolism due to a deficiency of fructose-1-phosphate aldolase (EC 126.96.36.199) activity, which results in an accumulation of fructose-1-phosphate in the liver, kidney, and small intestine. The accumulated fructose-1-phosphate inhibits glycogen breakdown and glucose synthesis, thereby causing severe hypoglycemia following ingestion of fructose.
Understandable Definition: HFI is a condition people are born with, usually in families where there is no previous history. The condition makes it difficult for the body, chiefly the liver and kidney, to use fructose and fructose-containing foods. The sugar is taken up by the body, but in attempting to use the sugar, toxic substances are produced, which lead to serious illness.
Symptoms: These include severe abdominal pain, vomiting, and hypoglycemia following ingestion of fructose or other sugars metabolized through fructose-1-phosphate. Prolonged fructose ingestion in infants leads ultimately to hepatic and/or renal failure and death. Patients develop a strong distaste for sweet food, and can avoid a recurrence of symptoms chronic course of the disease by remaining on a fructose- and sucrose-free diet.
What HFI is NOT: HFI symptoms are common to many other illness and disorders. However, HFI is most commonly confused with fructose malabsorption. This is a non-life threatening and much more common condition. A more complete comparison can be found here. The treatment for both is similar.
A more complete explanation of HFI can be found by clicking here.
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