Some problems at the genetic level provoke a violation of bilirubin metabolism. Because of this, pigmentary hepatosis can occur. Such diseases are considered quite dangerous. This is due to the fact that the patient has jaundice, but there are no problems with the liver of a viral, infectious type. Disorders of bilirubin metabolism are different, in accordance with them, and the main group of diseases is divided into several types.
Description
In the human body, erythrocytes live up to 120 days. After that, they split. One of the breakdown products is bilirubin. It is direct and indirect. This substance, after excretion, is processed in the liver and enters the intestines in the form of bile. If one of the stages is violated, an unprocessed substance remains in the blood. Because of this, the skin of a patient with pigmentary hepatosis becomes yellow.
Causes of disease
The described problem is hereditary. It occurs due to failures in the processing of bilirubin, which are transmitted togenetic level. At the moment, four types of problems are known. They are associated with etiological features:
- If bilirubin is sent to the blood, and not to bile, then we are talking about the occurrence of Dubin-Johnson syndrome.
- Rotor Syndrome has the above specifics, but its symptoms are weaker.
- Gilbert's pigmentary hepatosis occurs due to the fact that a person does not have enough of a substance that allows you to capture and remove bilirubin. The skin turns yellow. The concentration of bilirubin in the blood increases.
- With Crigler-Najjar syndrome, a person does not have the enzyme glucuronyl transferase in the body. There is a lot of bilirubin in the blood.
Symptomatics
What are the symptoms of pigmentary hepatosis, depends entirely on the exchange of bilirubin. Common manifestations are pain in the liver, yellowness of the skin and malfunctions in the production of bile. Consider the symptoms characteristic of each type of pathology:
- When the Dubin-Johnson syndrome develops, the patient will have yellow sclera. During pregnancy or when taking oral contraceptives in women, this manifestation may be aggravated. The secretion of bile is disturbed.
- With Rotor's disease, yellowness of the sclera occurs.
- Julbert's syndrome is pigmentary hepatosis, which is manifested by dull pain in the abdomen. The skin is yellow and the liver is enlarged.
- Crigler-Najjar disease is rare. Most often diagnosed immediately after birth. There is jaundice. If the pathology is severe, then problems begin withcentral nervous system. This often leads to the death of the patient. If the child has a mild form, then the skin turns a little yellow.
Hereditary pigmentary hepatosis can manifest itself in different ways. Let's look at some more symptoms. Sleep is disturbed, there is pain and heaviness in the liver, urine is darker, asthenic syndrome appears, manifested by dizziness and fatigue, appetite is lost, nausea occurs, the intestines begin to work poorly, the skin turns yellow and begins to itch intensely.
In order to make a diagnosis, it is also necessary to collect an anamnesis of relatives.
Disease diagnosis process
When diagnosing a problem, it is important to find out the true causes of yellowing of the skin, as well as to distinguish pigmentary hepatosis from a number of similar diseases. It is necessary to conduct a biochemical blood test. This will allow you to understand what the level of bilirubin in the blood is. You need to collect anamnesis. The person should talk about stress, nutrition, symptoms. Genetic studies are required. Given the similarity of the problem with viral hepatitis, tests should be done to refute or confirm its presence. Thanks to the ultrasound, you can understand what the condition of the liver is, as well as whether it has normal dimensions. Sometimes a liver biopsy is done. This will confirm the diagnosis.
Differential diagnostic methods
When making a diagnosis, it is important to distinguish other problems from hepatitis pigmentosa. Photo of people sufferingthis problem, are available in the article. Pathology can be confused with cirrhosis of the liver, viral hepatitis (occurring in both acute and chronic forms), persistent hepatitis (in a mild chronic form). Sometimes the symptoms are similar to hemolytic anemia.
Treating the problem
Given that the pathology is inherited, it is not possible to cure completely. Patients should avoid physical exertion, stress, and also choose the right place to work. You need to periodically add new vegetables and fruits to the diet in order to provide the body with all the reserves of the necessary substances. Every year, you will have to take a course of vitamin therapy twice. In particular, you need to pay attention to the B vitamins. They are needed more than any others.
Diet 5 should be followed for life. What does she mean? You should give up alcohol, fatty, sour, fried, as well as pastries, coffee, tea. If necessary, the doctor may prescribe therapy with hepatoprotective agents. The number of courses per year should not exceed 4, lasting 1 month.
Treating a specific syndrome
Treatment of pigmented hepatosis depends not only on the symptoms that a person has, but also on the type of problem:
- Crigler-Najjar syndrome. The disease is of the 1st and 2nd type. At the first, it is necessary to do a blood transfusion, as well as to carry out phototherapy. The second type involves the use of drugs with phenobarbital. Phototherapy is rarely prescribed. If a woman has jaundicebreast milk, it is better to switch to formula while feeding the baby.
- Julbert's syndrome is pigmentary hepatosis (ICD-10 code E80.4), which does not require special treatment. Most often, in patients, the processing of bilirubin is restored after a while on its own. If there is really a lot of it in the blood, then phenobarbital is prescribed. Doctors assure that such therapy does not negatively affect the human condition. However, morale begins to deteriorate. Most often, patients who have to take this drug for life begin to feel very sick, and depression develops against this background.
It must be remembered that treatment should be prescribed only after a complete diagnosis.
Restrictions in Gilbert's syndrome
Given that the problem is usually not treated with medication, some restrictions should be observed. This will get rid of the symptoms.
You can not eat a lot of food that overloads the stomach. This applies to fatty foods, as well as alcohol. Do not allow long breaks in eating. It also causes gastrointestinal problems. It is forbidden to professionally engage in sports and exercise yourself heavily. Insolation is prohibited. This is due to the fact that they enhance the skin tone. Physiotherapy that heats the liver area is prohibited. This may lead to a worsening of the condition. The syndrome may begin to progress rapidly.
If these recommendations are followed, the patient's living conditions will not deteriorate much.
Ursosan
"Ursosan" is considered one of the most effective means in the treatment of the problem described. It allows you to stabilize the condition of the membranes in the liver. This will reduce the permeability, as well as the impact on them from external factors. The remedy affects the amount of bile that is released into the intestine. The amount of bilirubin in the blood decreases. The drug helps prevent the destruction of liver cells by weakening the effects of toxins. Allows in the presence of cholesterol plaques to dissolve them. They can cure hepatitis C and B of the viral type, both chronic and acute form of the disease associated with gallstones, and cholangitis.
Positive reviews about this drug. It is an excellent tool, has no contraindications and performs well in therapy. Most often it is prescribed in the treatment of hepatosis Dubin-Johnson, Rotor, Krieger-Nayar.
Forecast and preventive measures
Unfortunately, due to the fact that hepatosis pigmentosa (according to ICD-10 code K76.9) is a hereditary disease, it is impossible to prevent it. The prognosis is most often favorable. Patients can live full lives. The only nuance that can bring discomfort is the yellow tint of the skin and sclera. However, it is easy to get used to it.
An unfavorable prognosis is only in the case of Crigler-Najjar hepatosis. Due to the huge amount of bilirubin in humans, intoxication begins. This affects the body as a whole, as well as the brain. Often, patients die withinthe initial stage of the problem.
Results
As mentioned above, due to the increased level of bilirubin in the blood, yellowing of the eyes and other mucous membranes, the patient's skin becomes uncomfortable. The consequences can be quite different: severe, leading to death, moderate, in which the symptoms can be temporarily stopped, and mild, not requiring special treatment. However, in all cases, hyperbilirubinemia will be considered a lifelong diagnosis.
People suffering from Gilbert's syndrome are not considered at risk, but they need to constantly undergo examinations and control the level of bilirubin in the blood. The treatment will bring the best effect only if the diagnosis is made quickly and the therapy is started on time.
