Short answer · Medically reviewed summary · Last updated: 2023-07-13
Hereditary Fructose Intolerance (HFI) is a rare genetic disorder that affects the body's ability to break down fructose, a type of sugar found in many fruits, vegetables, and sweeteners. Individuals with HFI lack an essential enzyme called aldolase B, which is responsible for metabolizing fructose in the liver. Symptoms: When individuals with HFI consume fructose, it builds up in their liver, leading to a range of symptoms.
What is Hereditary Fructose Intolerance
What is Hereditary Fructose Intolerance? Plain-language, medically reviewed definition plus the lived reality told by patients.
Hereditary Fructose Intolerance (HFI) is a rare genetic disorder that affects the body's ability to break down fructose, a type of sugar found in many fruits, vegetables, and sweeteners. Individuals with HFI lack an essential enzyme called aldolase B, which is responsible for metabolizing fructose in the liver.
Symptoms: When individuals with HFI consume fructose, it builds up in their liver, leading to a range of symptoms. These can include severe abdominal pain, nausea, vomiting, bloating, and low blood sugar levels. In infants, HFI may cause failure to thrive and developmental delays if left untreated.
Diagnosis: HFI is typically diagnosed through genetic testing or by conducting a fructose tolerance test. It is important to consult a healthcare professional for an accurate diagnosis.
Treatment: The primary treatment for HFI is a strict lifelong fructose-free diet. This involves avoiding foods and beverages containing fructose, sucrose, and sorbitol. With proper management, individuals with HFI can lead healthy lives.
Conclusion: Hereditary Fructose Intolerance is a genetic disorder that affects the body's ability to process fructose. It is crucial for individuals with HFI to follow a fructose-free diet to prevent symptoms and complications associated with the condition.
