About Fructose Intolerance, Hereditary

What is Fructose Intolerance, Hereditary?

Fructose intolerance, hereditary (HFI) is an inherited disorder caused by a deficiency of the enzyme fructose-1-phosphate aldolase. This enzyme is necessary for the breakdown of fructose, a sugar found in many fruits, vegetables, and honey. People with HFI cannot properly digest fructose, leading to a buildup of fructose in the body and a variety of symptoms. Symptoms of HFI can include abdominal pain, bloating, gas, nausea, and diarrhea. In severe cases, HFI can lead to liver damage. Treatment for HFI involves avoiding foods that contain fructose and other sugars, such as sucrose and sorbitol.

What are the symptoms of Fructose Intolerance, Hereditary?

Symptoms of fructose intolerance, hereditary, can vary from person to person, but may include:

- Abdominal pain

- Bloating

- Diarrhea

- Gas

- Nausea

- Vomiting

- Fatigue

- Headache

- Weight loss

- Poor appetite

- Irritability

- Dark urine

- Jaundice

- Weakness

- Rapid heart rate

- Low blood sugar (hypoglycemia)

- Anemia

What are the causes of Fructose Intolerance, Hereditary?

1. Hereditary fructose intolerance (HFI) is an inherited disorder caused by a deficiency of the enzyme fructose-1-phosphate aldolase. This enzyme is necessary for the metabolism of fructose, a simple sugar found in many fruits, vegetables, and honey.

2. Mutations in the gene that codes for the enzyme fructose-1-phosphate aldolase cause HFI. These mutations can be passed down from parent to child.

3. Other causes of fructose intolerance include certain medications, such as antibiotics, and certain medical conditions, such as celiac disease.

What are the treatments for Fructose Intolerance, Hereditary?

1. Avoidance of fructose: The most important treatment for fructose intolerance is to avoid foods that contain fructose. This includes foods such as apples, pears, honey, and high-fructose corn syrup.

2. Lactose-free diet: A lactose-free diet may also be recommended for people with fructose intolerance. This means avoiding dairy products, such as milk, cheese, and yogurt.

3. Fructose-free supplements: Fructose-free supplements may be recommended to help replace the fructose that is not being consumed.

4. Enzyme replacement therapy: Enzyme replacement therapy may be recommended for people with hereditary fructose intolerance. This involves taking a supplement that contains the enzyme fructose-1-phosphate aldolase, which helps break down fructose.

5. Dietary counseling: A dietitian

What are the risk factors for Fructose Intolerance, Hereditary?

1. Family history of fructose intolerance
2. Genetic mutations in the fructose transporter gene
3. Premature birth
4. Low birth weight
5. Gastrointestinal disorders
6. Liver disease
7. Diabetes
8. Hypothyroidism
9. Celiac disease
10. Food allergies

Is there a cure/medications for Fructose Intolerance, Hereditary?

There is no cure for fructose intolerance, hereditary. However, there are medications available to help manage the symptoms. These medications include lactase enzyme supplements, probiotics, and medications to reduce abdominal pain and bloating. It is important to speak with your doctor to determine the best treatment plan for your individual needs.