fruktózintoleranciának

Fruktózintolerancia, also known as hereditary fructose intolerance (HFI), is a rare inherited metabolic disorder that affects the body's ability to properly process fructose. Fructose is a type of sugar found naturally in fruits, honey, and some vegetables, and it is also used as a sweetener in many processed foods and drinks. In individuals with HFI, a deficiency in the enzyme aldolase B prevents the breakdown of fructose in the liver.

When someone with HFI consumes fructose, it accumulates in the liver, leading to a cascade of harmful effects. This buildup triggers the release of phosphate, which in turn depletes the body's stores of adenosine triphosphate (ATP), a crucial energy source for cells. The resulting hypoglycemia (low blood sugar) is a hallmark symptom, often appearing shortly after feeding infants or young children fructose-containing foods.

Symptoms of fructose intolerance can vary in severity but typically manifest in infancy or early childhood. These include nausea, vomiting, abdominal pain, diarrhea, poor growth, irritability, and in severe cases, liver damage and kidney problems. Prolonged exposure to fructose can lead to chronic liver disease and kidney failure.

Diagnosis of fructose intolerance is usually made through a combination of medical history, physical examination, and specific laboratory tests. A fructose tolerance test, where fructose is administered and blood glucose and other metabolic markers are monitored, can help confirm the diagnosis. Genetic testing is also available.

The management of fructose intolerance involves a strict, lifelong avoidance of fructose, sucrose (which breaks down into glucose and fructose), and sorbitol (a sugar alcohol that can be converted to fructose). This requires careful attention to dietary intake, including reading food labels and avoiding many processed foods and artificial sweeteners. With appropriate dietary management, individuals with fructose intolerance can lead healthy lives, but early diagnosis and consistent adherence to the restricted diet are essential.