Some problems at the genetic level provoke a violation of bilirubin metabolism. Because of this, pigmented hepatoses can occur. Such diseases are considered quite dangerous. This is due to the fact that the patient develops jaundice, but there are no problems with the liver of a viral, infectious type. Violations of bilirubin metabolism are various, in accordance with them the main group of diseases is divided into several types.
Description
In the human body, red blood cells live up to 120 days. After that, they split. One of the decay products is bilirubin. It can be direct and indirect. This substance, after isolation, is processed in the liver and enters the intestine in the form of bile. If one of the stages is violated, the unprocessed substance remains in the blood. Because of this, the skin of the patient with pigmented hepatosis becomes yellow.
Causes of the disease
The described problem is hereditary. It occurs due to disruptions in the processing of bilirubin, which are transmitted at the genetic level. Four types of problems are currently 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 Dabin-Johnson syndrome.
- Rotor syndrome has the specifics described above, but its symptoms are weaker.
- Gilbert's pigmented hepatosis occurs due to the fact that a person lacks a substance that allows it to capture and excrete bilirubin. The skin turns yellow. In the blood, the concentration of bilirubin increases.
- With Krigler-Nayyar syndrome, a person has no glucuronyl transferase enzyme in the body. There is a lot of bilirubin in the blood.
Symptomatology
What are the symptoms of pigmented hepatosis is completely dependent on bilirubin metabolism. 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:
- With the development of Dabin-Johnson syndrome, the patient will have yellow sclera. During pregnancy or when taking oral contraceptives in women, this manifestation may be exacerbated. Bile secretion is impaired.
- With Rotor disease, yellowness of the sclera occurs.
- Zhulber's syndrome - pigmented hepatosis, which is manifested by dull abdominal pain. The skin is yellow, and the liver is enlarged.
- Kriegler-Nayyar disease is rare. Most often diagnosed immediately after birth. Yellowness appears. If the pathology is severe, then problems begin with the central 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 pigmented hepatoses can manifest themselves in different ways. Consider some more symptoms. Sleep is disturbed, pain and heaviness occur in the liver, urine is darker, asthenic syndrome appears, which is manifested by dizziness and fatigue, appetite is lost, nausea occurs, the intestines begin to work poorly, the skin turns yellow and begins to scratch heavily.
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 pigmented hepatosis from a number of similar diseases. It is necessary to conduct a biochemical blood test. This will help to understand what is the level of bilirubin in the blood. Need to collect an anamnesis. A person should talk about stress, nutrition, symptoms. Be sure to conduct genetic research. Given the similarity of the problem with viral hepatitis, tests should be done and to refute or confirm its presence. Thanks to an ultrasound, you can understand what the condition of the liver is, as well as whether its size is normal. A liver biopsy is sometimes performed. This will confirm the diagnosis.
Differential diagnostic methods
When conducting diagnostics, it is important to distinguish other problems from pigmented hepatosis. Photos of people suffering from this problem are available in the article. Pathology can be confused with cirrhosis, viral hepatitis (occurring both in acute and in chronic form), persistent hepatitis (in mild chronic form). Sometimes the symptoms are similar to hemolytic anemia.
Problem treatment
Given that the pathology is inherited, it is not completely possible to recover. Patients should avoid physical exertion, stress, and also choose the right place of work. In the diet, you need to periodically add new vegetables and fruits to provide the body with all the supplies of the necessary substances. Each year, twice will have to undergo a course of vitamin therapy. In particular, you need to pay attention to the vitamins of group B. They are needed more than any other.
Diet number 5 should be followed all my life. What does she mean? You should abandon alcohol, fatty, sour, fried, as well as baking, coffee, tea. If necessary, the doctor may prescribe hepatoprotective therapy. The number of courses per year should not exceed 4, lasting 1 month.
Treatment for a specific syndrome
The treatment of pigmented hepatosis depends not only on the symptoms that a person has, but also on the type of problem:
- Krigler-Nayyar syndrome. The disease is of the 1st and 2nd type. At the first, it is necessary to do a blood transfusion, as well as conduct phototherapy. The second type involves the use of drugs with phenobarbital. Phototherapy is rarely prescribed. If a woman has jaundice of breast milk, then during feeding the baby it is better to transfer it to a mixture.
- Zhulber's syndrome - pigmented hepatosis (according to ICD-10 code E80.4), which does not require special treatment. Most often, in patients, bilirubin processing is restored through time on their own. If there is really a lot of it in the blood, then phenobarbital is prescribed. Doctors say that such a therapy is not negatively affected by a person’s condition. However, morale begins to deteriorate. Most often, patients who have to take this drug all their lives begin to feel very sick, and against this background, depression develops.
It must be remembered that treatment should be prescribed only after a complete diagnosis.
Limitations of Gilbert's Syndrome
Given that the problem is not usually 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. Long breaks in food intake should not be allowed. It also causes gastrointestinal problems. It is forbidden to professionally engage in sports and physically exert great strain on yourself. Insolation is prohibited. This is due to the fact that they enhance the skin tone. Physiotherapy that heats the liver area is prohibited. This can lead to deterioration. The syndrome can begin to progress rapidly.
If you follow these recommendations, the patient’s living conditions will not deteriorate much.
Ursosan
"Ursosan" is considered one of the most effective means in the treatment of the described problem. It allows you to stabilize the state of the membranes in the liver. Due to this, permeability is reduced, as well as the impact on them from external factors. The tool affects the amount of bile that is released into the intestine. In the blood, the amount of bilirubin is reduced. The drug helps prevent the destruction of liver cells by attenuating the effects of toxins. Allows in the presence of cholesterol plaques to dissolve them. They can treat hepatitis C and B of the viral type, both chronic and acute forms of the disease associated with gallstones, and cholangitis.
Positive feedback about this drug. It is an excellent tool, has no contraindications and is excellent in therapy. Most often, it is prescribed for the treatment of hepatoses of Dabin-Johnson, Rotor, Krieger-Nayyar.
Forecast and preventive measures
Unfortunately, due to the fact that pigmentary hepatosis (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 a full life. The only nuance that can bring discomfort is the yellow skin tone and sclera. However, this is easy to get used to.
An unfavorable prognosis is only in the case of Krigler-Nayyar hepatosis. Due to the huge amount of bilirubin in a person, intoxication begins. This affects the body as a whole, as well as the brain. Often patients die at the initial stage of the problem.
Summary
As already mentioned above, due to the increased level of bilirubin in the blood, yellowing of the eyes and other mucous membranes, the patient experiences discomfort. The consequences can be completely different: severe, leading to death, moderate, in which the symptoms can be temporarily stopped, and light, not requiring special treatment. However, in all cases, hyperbilirubinemia will be considered a lifelong diagnosis.
People suffering from Gilbert's syndrome are not considered a risk group, but they need to constantly undergo examinations and monitor the level of bilirubin in the blood. Treatment will bring the best effect only if you quickly diagnose and start therapy on time.