Dabin-Johnson Syndrome: Description, Diagnosis, Symptoms and Treatment

Despite the fact that modern medicine has moved far ahead and many diseases are no longer a sentence for patients, some of the diseases have not yet been studied to the end. One of them is the Dabin-Johnson syndrome. Clinic, diagnosis, treatment of this ailment is of interest to medical professionals. Let's consider this issue in more detail.

Rotor Syndrome and Dabin-Johnson syndrome are rare hereditary liver diseases , similar forms of jaundice.

Causes

The Dabin-Johnson syndrome also has another name: the enzyme jaundice of the liver. It arises in connection with the violation of delivery to the bile from the liver cells of bilirubin.

This substance appears in the body to remove from it damaged or completely destroyed red blood cells. The conclusion of bilirubin occurs in two ways:

• A greater amount of the substance exits through the bile;
• Small volumes go away with urine.

The Dabin-Johnson syndrome manifests itself when the transportation of bilirubin through bile is disrupted. This extremely rare disease is more likely to affect young men (approximately 20-30 years). Sometimes there are cases of the disease from birth. The risk of infection after reaching the age of 50 years and older is minimal.

The genetic reason for the occurrence of Dabin-Johnson syndrome is explained by the mutation of the gene involved in the export of bilirubin to the liver and encoding proteins.

Symptoms

Dabin-Johnson syndrome, the symptoms of which are inherent in other diseases, such as, for example, increased fatigue, has its own distinctive features:

• Pain in the ribs on the right side;
• Complete lack of appetite;
• There may be a slight itchy skin;
• Increased liver size;
• Colic, which are sometimes worse;
• Vomiting;
• Increase in temperature.

Manifestations intensify in connection with the transferred infectious diseases, heavy physical and emotional overloads. For women, exacerbation is possible after taking contraceptives.

Risk group

What group of people should be afraid of the syndrome?

The disease is hereditary. If the family has children with this disease, the pair during the pregnancy planning phase needs to undergo all the examinations, pass the necessary tests and consult with a specialist. The same recommendations are relevant for those couples, among whom some spouse or their immediate relatives have suffered the Dabin-Johnson syndrome.

Diagnostic process

Geographically, this disease is most common among Jews of Iranian origin. Their number reaches 70% of the total number of diseased patients.

It is important that Dabin-Johnson syndrome is practically not life-threatening. An example with patients confirms this. Doctors studied the average life expectancy of patients with this ailment and came to the conclusion that it does not lead to death.

About 25% of the patients experience pain in the right hypochondrium. The structure of the liver does not suffer from the syndrome, nor does it deform. But at the same time black spots appear in its tissues, as a result of which the organ changes its color to green or dark brown. This feature of the syndrome is called the chocolate liver. This effect is achieved as a result of a violation of the secretion of metabolites.

The disease has a favorable prognosis, despite the fact that it can continue throughout the life of the patient.

The table below shows the data on the condition of the patient's internal organs.

Instrumental diagnostics

The Dabin-Johnson syndrome, diagnosed in several stages, is determined by analysis.

• Obligatory ultrasound is the organs of the abdominal cavity.
• Common blood and urine tests.
• Analysis of blood and urine for bilirubin. As a rule, they are too high.
• Samples with phenobarbetal.
• Markers of hepatitis.
• Bromsulfalein test .
• The value of indicators of co-proporphyrin in urine per day.
• The content of enzymes in the blood.

Some other methods of diagnosing the disease are also used.

In the presence of Dabina-Johnson syndrome, the patient must always visit the therapist.

Treatment and prevention

Dabin-Johnson syndrome, whose treatment does not involve the use of special drugs, is controlled through preventive measures:

• Compliance with a strict diet, intake of B vitamins, refusal of preservatives;
• Reduction of stressful situations;
• Decreased physical activity;
• Refusal of alcoholic beverages;
• Regular monitoring of the level of bilirubin throughout the life of the patient.

It must also be remembered that pregnancy can exacerbate the disease. Drugs that help to cure Dabin-Johnson syndrome have not been developed at the moment.

A similar in nature disease is Rotor syndrome.

a brief description of

Rotor Syndrome is also a hereditary disease that affects the liver. Changes in the delivery of bilirubin, enzymatic functions remain normal. It can be said that the disease is a facilitated form of the Dabin-Johnson syndrome described above. Symptoms are also more mild.

The disease can occur both from birth and in childhood. Bilirubin in the patient's blood can be free and bound. There is contrasting of the gallbladder.

The syndrome of Rotor differs in that in hepatocytes there is no content of brown pigments. There is no effect, which is called the chocolate liver. The cause of the disease is slightly different from the Dabin-Johnson syndrome. In this case, the liver does not cope with the absorption of bilirubin. As a result of the failure, the undigested substance is returned back to the blood. On this soil in the patient's body develops hyperbulemia. The prognosis of the disease is favorable.

During the diagnosis of lidofrenin, the liver, gallbladder and its ducts become visible.

Symptoms of the syndrome

Symptoms of Rotor syndrome are very similar to those of Dabin-Johnson syndrome:

• Implicit jaundice.
• Sensation of bitterness in the mouth.
• Pain sensations in the right hypochondrium.
• With Dabin-Johnson syndrome, the patient's liver is enlarged in size. In the case of the Rotor Syndrome, it is increased only in some cases.
• Increased bilirubin content in the blood.
• The liver functions remain normal.
• Blood tests without changes.
• In the liver biopsy, no accumulation of pigments occurs.

The prognosis of the disease is generally favorable, the syndrome occurs over many years, not worsening the state of human health.

Diagnosis of the syndrome

The disease can be established only when carrying out a comprehensive diagnosis of the body.

Studies conducted in the laboratory will help to identify the high content of bilirubin in the blood. For diagnosis, it is important to know the amount of coproporphyrin contained in the urine.

In most patients, the disease proceeds without any symptoms.

In the case of diagnosis of the Rotor syndrome, a liver examination is performed : its X-ray and ultrasound. The gall bladder and ducts undergo the same analysis.

Prevention of disease

General prevention measures are as follows:

• Decreased physical activity;
• Treatment of diseases associated with Rotor syndrome;
• You should avoid stress and emotional stress.

Factors of increased bilirubin in the blood

Bilirubin is increased for the following reasons:

• Severe stress;
• Malnutrition, violation of diet;
• Drug use;
• Excessive use of alcohol.

Treatment of the disease

There are no common methods for eliminating the disease. However, preventive measures must be followed. As drugs, you need to take funds that remove bilirubin from the body. Cholagogue preparations are often used.

Find a suitable treatment will help a doctor.