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Avoid fructose-containing formulas in infants with hereditary fructose intolerance

Avoid fructose-containing formulas in infants with hereditary fructose intolerance
Infants with hereditary fructose intolerance (HFI) have a high risk of developing life-threatening acute liver failure if they are fed with widely available fructose-containing formulas, according to a new study published in the journal Molecular Genetics and Metabolism.
Hong Li, assistant professor of human genetics and pediatrics at Emory University School of Medicine, and colleagues reported four cases of HFI, all diagnosed in early infants. All had acute liver failure that resolved when the infants switched to formula without fructose.
Hereditary fructose intolerance is an autosomal recessive disorder caused by the deficiency of aldolase B (ALDOB) resulting in impaired ability to metabolize fructose. The toxic accumulation of intermediate fructose-1-phosphate causes multiple metabolic disturbances including lactic acidosis,  liver/kidney dysfunction, electrolyte disturbance and postprandial hypoglycemia. Early symptoms of HFI include vomiting, nausea, abdominal pain and failure to gain weight. The condition is estimated to occur in 1 out of 20,000 live births.

“In some of the cases we describe in this article, the treating physician had to call the formula manufacturer to confirm that sucrose was a component,” says Li. “It underlines why accurate and explicit labeling is necessary.”

HFI is, in a sense, a mirror image of galactosemia, an inherited intolerance of lactose which Li says is better known.

“Most pediatricians are aware of galactosemia, and the liver symptoms that may bring an infant to see a doctor actually resemble galactosemia, even if they come from a different origin,” she says. “In the case of HFI, choosing formulas that avoid lactose, like soy-based formulas containing fructose or sucrose, can make things worse.”

In two of the cases, there was a family history that suggested a risk of inherited metabolic disorders, but a precise diagnosis was not available. Doctors thought one of the cases looked like a mitochondrial disorder. Another was initially suspected to be an infection due to the combination of acute liver failure, hypoglycemia and bacteria growth in the urine.

Since HFI is a treatable disease, Li urges pediatricians to consider HFI as a potential diagnosis if there is a feeding problem, elevated transaminase enzymes or jaundice (a sign of liver damage) and the infant has been fed formula containing fructose or sucrose.

The only way to confirm HFI is by genetic sequencing, however, some information regarding the condition can be obtained by urine carbohydrate tests. In any case, the solution is simple: avoid foods and formulas containing fructose and sucrose.

“Baby formula manufacturers should remove fructose or sucrose, or explicitly label their products to allow parents to avoid those sweeteners if necessary,” suggests the researchers.

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Source: With inputs from MGM

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