Sugars in infant formulas pose risk to babies with inherited metabolic disorder

May 16, 2018 by Quinn Eastman, Emory University
Sugars in infant formulas pose risk to babies with inherited metabolic disorder
Babies with inherited intolerance of fructose face a risk of acute liver failure if they are fed certain formulas containing sucrose or fructose, pediatricians and geneticists are warning. Credit: Emory University

Babies with inherited intolerance of fructose face a risk of acute liver failure if they are fed certain widely available formulas containing fructose, pediatricians and geneticists are warning. Baby formula manufacturers should remove fructose or sucrose, or explicitly label their products to allow parents to avoid those sweeteners if necessary, the doctors say.

In a recent paper in Molecular Genetics and Metabolism, Emory geneticists Hong Li, MD, Ph.D. and Michael Gambello, MD, Ph.D. together with Children's Healthcare of Atlanta pediatric hepatologist Miriam Vos, MD and colleagues report four cases of hereditary intolerance (HFI), all diagnosed in early infants. All had acute liver failure that resolved when the infants switched to formula without fructose.

HFI is estimated to occur in 1 out of 20,000 live births. It comes from mutations in the aldolase B gene, resulting in an inability to metabolize fructose. Early symptoms include nausea, vomiting, abdominal pain and failure of an infant to gain weight. If unrecognized, HFI can result in liver and kidney damage, seizures or death.

HFI-related problems do not appear if an infant is being breastfed exclusively. It is normally recognized when fructose-containing solid foods, such as fruit, are introduced into the diet several months after birth. However, some baby formulas – often soy-based – contain sweeteners such as high-fructose corn syrup or sucrose (table sugar), which is made of fructose and glucose linked together. Sometimes, the label only says "sugar" instead of sucrose.

"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, assistant professor of human genetics and pediatrics at Emory University School of Medicine. "It underlines why accurate and explicit labeling is necessary."

In babies with digestive problems or allergies, parents or pediatricians may seek to avoid formulas based on cows' milk. However, in alternative formulas, there is a higher chance the manufacturer will add sucrose or fructose as a sweetener and carbohydrate source.

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."

Gambello, associate professor of human genetics and pediatrics at Emory University School of Medicine and Section Chief of the Division of Medical Genetics, notes that HFI occurs frequently enough that several babies can be expected to be born in Georgia every year with the condition. The paper reports on two cases from Georgia and two others from Ohio and California.

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 , 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.

Some information about a young patient's condition can be obtained from urine carbohydrate tests, but the only way to confirm HFI is by genetic sequencing. In any case, the solution is simple: avoid foods and formulas containing fructose and .

"Once parents switch formula, improvement happens very fast," Li says.

Explore further: Metastatic cancer gorges on fructose in the liver

More information: Hong Li et al. Acute liver failure in neonates with undiagnosed hereditary fructose intolerance due to exposure from widely available infant formulas, Molecular Genetics and Metabolism (2018). DOI: 10.1016/j.ymgme.2018.02.016

Related Stories

Metastatic cancer gorges on fructose in the liver

April 26, 2018
Biomedical engineers at Duke University have demonstrated that metastatic cancer cells can reprogram their metabolism to thrive in new organs. Specifically, the research shows that cells originating from colorectal cancer ...

High-fructose diet during and after pregnancy can cause a fatty liver in offspring

April 27, 2017
A diet high in fructose-containing sugars eaten during pregnancy or while breastfeeding can cause offspring to have a fatty liver, increasing their chances of developing obesity or type 2 diabetes. This is according to a ...

Researchers link obesity and the body's production of fructose

September 10, 2013
Researchers at the University of Colorado School of Medicine reported today that the cause of obesity and insulin resistance may be tied to the fructose your body makes in addition to the fructose you eat.

New study finds no reason to replace fructose with glucose

January 31, 2014
Researchers at St. Michael's Hospital have found there is no benefit in replacing fructose, the sugar most commonly blamed for obesity, with glucose in commercially prepared foods.

New study finds neither HFCS nor table sugar increases liver fat under 'real world' conditions

February 12, 2013
A study published in the Journal of Applied Physiology, Nutrition, and Metabolism presented compelling data showing the consumption of both high fructose corn syrup (HFCS) and sucrose (table sugar) at levels consistent with ...

Fructose not responsible for increase in non-alcoholic fatty liver disease

February 26, 2014
Non-alcoholic fatty liver disease is the most common chronic liver disease in developed countries, affecting up to 30 per cent of their populations.

Recommended for you

Analytical tool predicts genes that can cause disease by producing altered proteins

July 19, 2018
Predicting genes that can cause disease due to the production of truncated or altered proteins that take on a new or different function, rather than those that lose their function, is now possible thanks to an international ...

Childhood stress leaves lasting mark on genes

July 18, 2018
Kids who experience severe stress are more likely to develop a host of physical and mental health problems by the time they reach adulthood, including anxiety, depression and mood disorders. But how does early life stress ...

Study shows DNA methylation related to liver disease among obese patients

July 18, 2018
DNA methylation is a molecular process that helps enable our bodies to repair themselves, fight infection, get rid of environmental toxins, and even to think. But sometimes this process goes awry.

Protein found to be key component in irregularly excited brain cells

July 17, 2018
In a new study in mice, researchers have identified a key protein involved in the irregular brain cell activity seen in autism spectrum disorders and epilepsy. The protein, p53, is well-known in cancer biology as a tumor ...

World's largest study on allergic rhinitis reveals new risk genes

July 17, 2018
An international team of scientists led by Helmholtz Zentrum München and University of Copenhagen has presented the largest study so far on allergic rhinitis in the journal Nature Genetics. The data of nearly 900,000 participants ...

New platform poised to be next generation of genetic medicines

July 16, 2018
A City of Hope scientist has discovered a gene-editing technology that could efficiently and accurately correct the genetic defects that underlie certain diseases, positioning the new tool as the basis for the next generation ...

0 comments

Please sign in to add a comment. Registration is free, and takes less than a minute. Read more

Click here to reset your password.
Sign in to get notified via email when new comments are made.