Hereditary fructose intolerance is a rare disorder in which the body is not able to break down fructose due to lack of liver enzymes. This article describes the symptoms, diagnosis, and treatment options for this condition.
Fructose is a simple sugar that can be naturally found in the body, it is also found in fruits and honey. The enzyme which is needed to break down this simple sugar is called Aldolase B. When the liver is unable to produce this enzyme, it can lead to a disorder known as hereditary fructose intolerance. As the body is not able to break down fructose, it accumulates in various parts of the body like in the intestine, liver, and kidneys. This accumulation curbs the breakdown or synthesis of glycogen. This can also lead to hypoglycemia, as sugar levels in the blood fall considerably due to lack of glucose. This is caused by an abnormal mutated gene which is inherited from the parents. When both the parents carry one abnormal mutated gene each, their children have a twenty five percent chance of acquiring this disorder. This condition can cause severe health hazards, so it’s important to know its symptoms and detect them early.
Symptoms
- Vomiting
- Uncontrollable contraction of muscles
- Excessive sleepiness
- Abdominal Pain
- Jaundice
- Irritation after eating fructose rich foods
- Strong disliking for sweet food
If these symptoms are ignored or left untreated, it may lead to severe disorders of the liver and kidneys. In some cases, it can also lead to kidney failure. So, early diagnosis of this disease is necessary.
Diagnosing the Condition
Blood clotting and blood sugar tests can help determine low levels of blood sugar. Even liver biopsy can be helpful for finding the cause of liver enlargement and abnormal levels of enzymes. A test is also carried out wherein fructose is injected in the body and levels of glucose, fructose, and phosphate are checked and monitored by taking blood samples. However, this test can be risky, as increased fructose levels can be hazardous. Some definite tests are an enzyme test and a DNA test.
Enzyme Test: A sample of enzymes is obtained from the liver during biopsy and is tested. Enzyme test is definite because the analysis reveals whether there is a presence of enzyme Aldolase B in the liver, and if present, is it functioning properly?
DNA Test: Since this condition is caused due to a mutated gene, DNA testing can help in determining this disorder by analyzing the defect or abnormality of certain genes.
Treatment Options
The only effective treatment for this condition is a fructose free diet. For such a diet one should exclude sugar, honey, sugar coated cereals, sweet potatoes, carrot, tomato, mayonnaise, milk products containing sugar, desserts, fruits, and fruit juices from his daily meals. Foods which can be included in this diet are meat, eggs, fish, vegetables, nuts, and rice.
People with a family history of this disease should consult a doctor for the early diagnosis. About one in ten thousand people suffer from this health condition, so it is a very rare disease. Due to the nature of treatment involved, a person suffering from this condition has to adopt a new way of living. A fructose free diet will keep you away from many critical diseases and will lead to a normal and healthy life.
