Fructosuria

Fructosuria or Levulesauri

**Fructosuria**, also known as fructosemia, fructosenuria, levulosis uri, etc., is a condition in which a fair amount of fructose is present in the urine - organic monosaccharide compounds that can dissolve in water. And the name of the pathology is quite true. This is a fairly rare hereditary metabolic disorder characterized by excessive excretion of fructose in the urine.

Often, doctors use the term “fructosemia” to designate this pathology. In some sources, the same situation is called levulosauria, although these two concepts have no basis. The word “glucose mania” has nothing to do with glucose and is found in only one source. Now, according to the existing classification of diseases, these concepts are considered synonymous. However, to simplify the description of the condition, we will use the word fructose

Fructosuria: understanding and consequences of excess fructose

Fructosuria, also known as fructosemia or fructosaurinia, is a rare genetic disorder of carbohydrate metabolism that is characterized by impaired metabolism of fructose. This condition is caused by the presence of a defective enzyme, fructose-1-phosphate aldolase, which is unable to completely metabolize fructose. In the presence of fructosuria, fructose cannot be converted into glucose, which leads to its accumulation in the body.

The cause of fructosuria is associated with an inherited defect in the gene responsible for the production of fructose-1-phosphate aldolase. This enzyme plays a key role in the breakdown of fructose, and its deficiency leads to the accumulation of fructose in the blood and tissues.

One of the most common symptoms of fructosuria in newborns is increased fatigue, irritability and refusal to feed. Older children and adults may experience recurring episodes of nausea, vomiting, abdominal pain, and general weakness. Long-term disruption of fructose metabolism can lead to serious complications such as liver damage, psychomotor retardation, and renal dysfunction.

Laboratory methods are used to diagnose fructosuria, including urine and blood tests for the presence of fructose and other metabolites. Early diagnosis is an important aspect of managing this condition to prevent serious complications.

Treatment of fructosuria involves strict adherence to a diet that excludes the consumption of fructose and other sources of fructose, such as sugar, syrups, honey and fruit juices. Limiting fructose consumption helps prevent its accumulation in the body and reduce the risk of complications.

Despite the fact that fructosuria is a rare disease, knowledge about it is important both for medical professionals and for society as a whole. Understanding the causes, symptoms and consequences of excess fructose can help diagnose and effectively manage this metabolic disorder.

In conclusion, fructosuria is a genetic disorder of carbohydrate metabolism that results in the accumulation of fructose in the body. Early diagnosis and strict adherence to a fructose-free diet are key measures to effectively manage fructosuria and prevent possible complications. More in-depth research in this area could help develop new diagnostic and treatment methods, improving the quality of life for patients suffering from this rare genetic disorder.