Other disorders of fructose metabolism Save

Other Disorders of Fructose Metabolism

Fructose is a simple sugar that is found naturally in fruits, vegetables, and honey. It is also added as a sweetener to many processed foods and drinks. When we consume fructose, our body breaks it down and uses it as a source of energy. However, some people have a genetic disorder that affects their ability to metabolize fructose properly. This can lead to a variety of health problems.

1. Fructose Intolerance: This is the most common disorder of fructose metabolism. People with this condition have a deficiency of an enzyme called aldolase B, which is needed to break down fructose in the liver. When they consume fructose, it builds up in their liver and causes symptoms such as abdominal pain, bloating, and diarrhea.
2. Hereditary Fructose Intolerance: This is a rare genetic disorder that affects the same enzyme as fructose intolerance. However, in this case, the deficiency is much more severe and can cause serious liver and kidney damage if left untreated. Symptoms include vomiting, jaundice, and hypoglycemia.
3. Essential Fructosuria: This is a benign disorder that affects the way the body absorbs and uses fructose. People with this condition can't convert fructose into glucose, but it doesn't cause any harmful effects. Symptoms are usually mild and include bloating and gas.

Other disorders of fructose metabolism include glucose transporter type 2 deficiency syndrome, which affects the way the body absorbs glucose and fructose, and phosphoglucomutase deficiency, which affects the breakdown of glycogen into glucose.

It is important for people with these disorders to avoid consuming fructose and to follow a strict diet. They may also need to take medication to manage their symptoms. If you suspect that you or your child has a disorder of fructose metabolism, it is important to speak with a healthcare professional for diagnosis and treatment.