Hereditary fructose intolerance
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Hereditary fructose intolerance

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Published: July 27, 2026 | Last Reviewed: July 27, 2026 | Medically Reviewed by: Dr. Sarah Mitchell, MD, Geriatrics | Reading Time: 5 min




Key Takeaways

  • Understanding hereditary fructose intolerance seniors metabolic disorder diet is important for seniors and their caregivers.
  • Symptoms can be seen after a baby starts eating food or formula.
  • Physical examination may show:
  • Removing fructose and sucrose from the diet is an effective treatment for most people.
  • Hereditary fructose intolerance may be mild or severe.

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What Are the Common Causes and Risk Factors?

Understanding hereditary fructose intolerance seniors metabolic disorder diet is important for seniors and their caregivers. this condition occurs when the body is missing an enzyme called aldolase B. This substance is needed to break down fructose.

If a person without this substance eats fructose or sucrose (cane or beet sugar, table sugar), complicated chemical changes occur in the body. The body cannot change its stored form of sugar (glycogen) into glucose. As a result, blood sugar falls and dangerous substances build up in the liver.

Hereditary fructose intolerance is inherited, which means it can be passed down through families. If both parents carry a nonworking copy of the aldolase B gene, each of their children has a 25% (1 in 4) chance of being affected. This is called autosomal recessive inheritance.

What Are the Symptoms and Warning Signs?

Symptoms can be seen after a baby starts eating food or formula.

The early symptoms of fructose intolerance are similar to those of

galactosemia

(inability to use the sugar galactose). Later symptoms relate more to liver disease.

Symptoms may include:

  • Convulsions

  • Excessive sleepiness

  • Irritability

  • Yellow skin or whites of the eyes (jaundice)
  • Poor feeding and growth as a baby,

    faltering weight

  • Problems after eating fruits and other foods that contain fructose or sucrose

  • Vomiting

📖 What Are Exams and Tests?

What Are Exams and Tests?

Physical examination may show:

  • Enlarged liver

    and

    spleen

  • Jaundice

Tests that confirm the diagnosis include:

  • Blood clotting tests

  • Blood sugar test

  • Enzyme studies
  • Genetic testing

  • Kidney function tests

  • Liver function tests

  • Liver biopsy

  • Uric acid blood test

  • Urinalysis

Blood sugar will be low, especially after receiving fructose or sucrose. Uric acid levels will be high.

📖 How Is It Treated?

How Is It Treated?

Removing fructose and sucrose from the diet is an effective treatment for most people. Complications may be treated. For example, some people can take a medicine to lower the level of uric acid in their blood and decrease their risk for

gout

.

📖 What Is Outlook (Prognosis)?

What Is Outlook (Prognosis)?

What Is Outlook (Prognosis)? - illustration

Hereditary fructose intolerance may be mild or severe.

Avoiding fructose and sucrose helps most children with this condition. The prognosis is good in most cases.

A few children with a severe form of the disease will develop severe liver disease. Even removing fructose and sucrose from the diet may not prevent severe liver disease in these children.

How well a person does depends on:

  • How soon the diagnosis is made
  • How soon fructose and sucrose can be removed from the diet
  • How well the enzyme works in the body
📖 What Are Possible Complications?

What Are Possible Complications?

These complications may occur:

  • Avoidance of fructose-containing foods due to their effects
  • Bleeding
  • Gout
  • Illness from eating foods containing fructose or sucrose
  • Liver failure
  • Low blood sugar (

    hypoglycemia

    )

  • Seizures

  • Death
📖 When to Contact a Medical Professional?

When to Contact a Medical Professional?

Contact your health care provider if your child develops symptoms of this condition after feeding starts. If your child has this condition, experts recommend seeing a doctor who specializes in biochemical genetics or metabolism.

📖 How Is It Prevented?

How Is It Prevented?

How Is It Prevented? - illustration

Couples with a family history of fructose intolerance who wish to have a baby may consider

genetic counseling

.

Most of the damaging effects of the disease can be prevented by decreasing fructose and sucrose intake.

Fructosemia; Fructose intolerance; Fructose aldolase B-deficiency; Fructose-1, 6-bisphosphate aldolase deficiency

Hijazi G, Kishnani PS. Defects in metabolism of carbohydrates. In: Kliegman RM, St. Geme JW, Blum NJ, et al, eds.

Nelson Textbook of Pediatrics.

22nd ed. Philadelphia, PA: Elsevier; 2025:chap 107.

Mojica A, Weinstock RS. Carbohydrates. In: McPherson RA, Pincus MR, eds.

Henry’s Clinical Diagnosis and Management by Laboratory Methods.

24th ed. Philadelphia, PA: Elsevier; 2022:chap 17.

Pearl PL, DiBacco ML, Gibson KM. Inborn errors of metabolism and the nervous system. In: Jankovic J, Mazziotta JC, Pomeroy SL, Newman NJ, eds.

Bradley and Daroff’s Neurology in Clinical Practice.

8th ed. Philadelphia, PA: Elsevier; 2022:chap 91.

Scheinman SJ. Genetically based kidney transport disorders. In: Gilbert SJ, ed.

National Kidney Foundation’s Primer on Kidney Disease.

8th ed. Philadelphia, PA: Elsevier; 2023:chap 37.

Updated by: Anna C. Edens Hurst, MD, MS, Associate Professor in Medical Genetics, The University of Alabama at Birmingham, Birmingham, AL. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team. Editorial update 06/17/2026.


  • Carbohydrate Metabolism Disorders

Frequently Asked Questions

Key Takeaways

Understanding hereditary fructose intolerance seniors metabolic disorder diet is important for seniors and their caregivers. Symptoms can be seen after a baby starts eating food or formula. Physical examination may show:
Removing fructose and sucrose from the diet is an effective treatment for most people.

What Are the Common Causes and Risk Factors?

Understanding hereditary fructose intolerance seniors metabolic disorder diet is important for seniors and their caregivers. this condition occurs when the body is missing an enzyme called aldolase B. This substance is needed to break down fructose.

What Are the Symptoms and Warning Signs?

Symptoms can be seen after a baby starts eating food or formula. The early symptoms of fructose intolerance are similar to those of

galactosemia

(inability to use the sugar galactose). Later symptoms relate more to liver disease.

What Are Exams and Tests?

Physical examination may show:

Enlarged liver

and

spleen

Jaundice

Tests that confirm the diagnosis include:

Blood clotting tests

Blood sugar test

Enzyme studies

Genetic testing

Kidney function tests

Liver function tests

Liver biopsy

Uric acid blood test

Urinalysis

Blood sugar will be low, especially after receiving fructose or sucrose. Uric acid levels will be high. 📖 How Is It Treated?

How Is It Treated?

Removing fructose and sucrose from the diet is an effective treatment for most people. Complications may be treated. For example, some people can take a medicine to lower the level of uric acid in their blood and decrease their risk for

gout

.

About the Author: This article was researched and written by the SilverWell Hub editorial team. It was medically reviewed by Dr. Sarah Mitchell, MD, Geriatrics.

Sources: This article is adapted from MedlinePlus, a service of the National Library of Medicine.

Disclaimer: This information is for educational purposes only. See our full Medical Disclaimer.

Published: July 27, 2026 | Next review: January 2027