For most individuals, consuming a diverse range of fruits and vegetables is fundamental to maintaining a healthy diet. However, for those with hereditary fructose intolerance, even a small amount of watermelon or sun-dried tomatoes can lead to significant health issues.
What is Hereditary Fructose Intolerance?
Hereditary fructose intolerance is a rare genetic disorder that affects the body’s ability to process fructose, a sugar found not only in fruits but also in honey, certain vegetables, sweetened beverages, and many packaged foods like cakes, cookies, sauces, and some breads. Fructose is also present in some processed meats and dairy products. Additionally, sucrose (table sugar) and sorbitol (a sugar substitute found in chewing gum, toothpaste, and medications) contain fructose or are converted into fructose during digestion. This means individuals with hereditary fructose intolerance must avoid these sugars as well.
The Genetic Basis of the Condition
The condition is caused by a deficiency of the enzyme aldolase B, which is crucial for breaking down fructose. Without this enzyme, fructose accumulates in the liver, kidneys, and intestines, potentially leading to severe health problems such as seizures, coma, and even death due to liver and kidney failure. Hereditary fructose intolerance is inherited when both parents carry the gene, affecting approximately one in 10,000 people.
Symptoms and Diagnosis
Symptoms of hereditary fructose intolerance typically become apparent when infants start consuming solid foods that contain fructose, such as fruits, vegetables, or sweetened baby foods. In adults, the condition can be misdiagnosed as other disorders like glycogen storage disease, eating disorders, or recurrent hepatitis, due to overlapping symptoms. This can result in the condition remaining undetected for years.
In infants and young children, symptoms may include vomiting, unusual sleepiness or irritability, food refusal, and failure to gain weight. Some children may instinctively avoid sweet foods, which can delay diagnosis until later in life. In adults, symptoms can include chronic stomach pain, fatigue, and unexplained low blood glucose levels. Doctors might notice signs such as a swollen liver, abnormal liver tests, or indications of fatty liver disease.
Confirming hereditary fructose intolerance requires genetic testing or a specialized glucose tolerance test. However, many individuals only receive a diagnosis after years of confusion and dietary experimentation.
Managing Hereditary Fructose Intolerance
There is no cure for hereditary fructose intolerance, but it can be managed by strictly avoiding fructose, sucrose, and sorbitol. Reading food labels becomes essential, as these sugars can be present in sauces, medications, and even toothpaste. Awareness of this rare condition can lead to earlier diagnosis and better support for those affected.
For parents, noticing a child’s sudden aversion to sweets, repeated vomiting, or slow growth can be crucial indicators. For healthcare professionals, considering hereditary fructose intolerance as a potential cause of unexplained digestive issues, low blood glucose, or liver changes can make a significant difference in a patient’s life.
Conclusion
Hereditary fructose intolerance is a rare genetic disorder that requires careful dietary management to prevent serious health complications. Greater awareness and understanding of this condition can lead to earlier diagnosis and improved quality of life for those affected.
For more information on hereditary fructose intolerance, including recipes, tips on reading food labels, and support, visit [HFI Support](https://hfisupport.org.au/).
🔗 **Fuente:** https://medicalxpress.com/news/2025-11-intolerant-fruit-veg.html