Hereditary Fructose Intolerance

Fructose is simple sugar, which is naturally found in the body, it is also found in fruits and honey. The enzyme which is needed to break down fructose is 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, the excess fructose accumulates in various parts of the body like the intestine, liver and kidneys. Accumulation of fructose curbs the breakdown or synthesis of glycogen. This can also lead to hypoglycemia, as sugar levels in the blood falls considerably, due to lack of glucose. This is caused by an abnormal mutated gene, which is inherited from parents. When both the parents carry one abnormal mutated gene each, their children have a twenty five percent chance of acquiring the disease. This condition can cause severe health hazards, so it's important to know the symptoms of this condition and detect them early.

Symptoms

During this time, the body is not able to break down fructose, which leads to build up of harmful substances in the liver. Let's look at some 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 kidney. In some cases, it can also lead to kidney failure. So, early diagnosis of this disease is necessary.

Diagnosing the Condition

Blood tests like blood clotting test, blood sugar test can help to determine low levels of blood sugar. Even liver biopsy can be helpful to find out the cause of liver enlargement and abnormal levels of enzymes. The 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 DNA test.

Enzyme Test
A sample of enzymes is obtained from liver tissues taken during liver biopsy. Enzyme test is definite because the analysis reveals whether there is a presence of enzyme aldolase B 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

Jaundice, liver and kidney disorders can also occur which can be treated individually through different medications, but the only effective treatment is a fructose free diet. To follow a diet free from fructose and sucrose is inevitable, as it will help to avoid any further health complications. A person following proper diet should exclude sugar, honey, sugar coated cereals, sweet potatoes, carrot, tomato, mayonnaise, milk products containing sugar, desserts, fruits, and fruit juices. Foods which can be included in the diet are meat, eggs, fish, vegetables, nuts, and rice.

Parents having a family history of this disease should consult a doctor for the early diagnosis and safety of their baby. Patients are also advised to have regular check-ups. About one in ten thousand 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 learn to adopt a new way of living. A fructose free diet will help to stay away from any critical diseases and lead a normal healthy life.