Despite the fact that modern medicine has stepped far forward and many diseases are no longer a sentence for patients, some of the diseases have not yet been fully studied. One of them is Dabin-Johnson syndrome. The clinic, diagnosis, treatment of this ailment is of particular interest for doctors. Let's consider this question in more detail.
Rotor syndrome and Dabin-Johnson syndrome are rare hereditary diseases of the liver, similar forms of jaundice.
Causes of occurrence
Dabin-Johnson syndrome also has another name: enzymatic jaundice of the liver. It arises in connection with a violation of the delivery of bilirubin from liver cells to bile.
This substance appears in the body to remove damaged or completely destroyed red blood cells from it. Bilirubin withdrawal occurs in two ways:
- a larger amount of substance leaves through bile;
- small volumes go with urine.
Dabin-Johnson syndrome manifests itself when the transport of bilirubin through bile is disrupted. This extremely rare disease is more prone to young men (from about 20-30 years old). Sometimes there are cases of the onset of the disease from birth. The risk of infection after a person reaches the age of 50 years and older is minimal.
The genetic cause of Dabin-Johnson syndrome is due to a mutation in a gene involved in the export of bilirubin to the liver and encoding proteins.
Symptoms
Dabin-Johnson syndrome, the symptoms of which are also characteristic of other diseases, such as increased fatigue, has its own distinguishing features:
- pain in the ribs on the right side;
- complete lack of appetite;
- slight itching of the skin may be observed;
- enlargement of the liver;
- colic, which sometimes intensifies;
- vomiting
- increase in temperature.
Manifestations are enhanced in connection with the infectious diseases, severe physical and emotional overload. For women, exacerbation is possible after taking contraceptives.
Risk group
Which group of people should be wary of the syndrome?
The disease is hereditary. If children with this disease were born in the family, the couple at the stage of pregnancy planning must undergo all examinations, pass the necessary tests and consult with a specialist. The same recommendations are relevant for those couples, among whom some spouses or their immediate family suffered Dabin-Johnson syndrome.
Diagnostic process
Geographically, this disease is most common among Jews of Iranian origin. Their number reaches 70% of the total mass of sick patients.
It is important that Dabin-Johnson syndrome is practically not life-threatening. The patient example confirms this. Doctors studied the average life expectancy of patients with this disease and came to the conclusion that it does not lead to death.
About 25% of patients experience pain in the right hypochondrium. The structure of the liver in the syndrome does not suffer, its deformation does not occur. But at the same time black spots appear in her tissues, as a result of which the organ changes its color to green or dark brown. This feature of the syndrome is called chocolate liver. This effect is achieved as a result of impaired metabolite secretion.
The disease has a favorable prognosis, despite the fact that it can continue throughout the life of the patient.
The table below reflects the status of the internal organs of patients.
| Organ | condition |
| liver | usually increased |
| gall bladder and ducts | not changed |
| spleen | often increased
|
Instrumental diagnostics
Dabin-Johnson syndrome, the diagnosis of which takes place in several stages, is determined by analysis.
- Ultrasound of the abdominal organs is mandatory.
- General blood and urine tests.
- Blood and urine tests for bilirubin. They are usually overpriced.
- Samples with phenobarbetal.
- Markers of hepatitis.
- Sample bromsulfaleinova.
- The value of coproporphyrin in urine per day.
- The content of enzymes in the blood.
Some other methods of diagnosing the disease are also used.
| Title | Characteristic |
| diagnostic laparoscopy | liver darkening characteristic for this syndrome |
| puncture biopsy | the presence of specific pigment in hepatic hepatocytes |
| intravenous cholecystography procedure | contrast of the gallbladder and its ducts is delayed or not carried out at all |
In the presence of Dabin-Johnson syndrome, the patient must visit a therapist.
Treatment and prevention
Dabin-Johnson syndrome, the treatment of which does not involve the use of special drugs, is controlled by preventive measures:
- adherence to a strict diet, the use of B vitamins, the rejection of preservatives;
- stress reduction;
- reduction of physical activity;
- refusal of alcoholic beverages;
- regular monitoring of bilirubin throughout the patient’s life.
It must also be remembered that pregnancy can exacerbate the disease. Medicines that help cure Dabin-Johnson syndrome have not yet been developed.
A similar disease is Rotor syndrome.
a brief description of
Rotor Syndrome is also a hereditary disease that affects the liver. Delivery of bilirubin undergoes changes, enzymatic functions remain normal. It can be said that the disease is a lightened form of the Dabin-Johnson syndrome described above. Symptoms are also slightly milder.
The disease can occur both from birth and in childhood. Bilirubin in the patient’s blood can be free and bound. There is a contrast of the gallbladder.
Rotor syndrome is characterized in that there are no brown pigment contents in hepatocytes. There is no effect called chocolate liver. The cause of the disease is slightly different from 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 basis, hyperbulemia develops in the patient's body. The prognosis of the disease is favorable.
During the diagnosis of lidophrenin, the liver, gall bladder and its ducts become visible.
Symptoms of the Syndrome
The signs of Rotor syndrome are very similar to the symptoms of Dabin-Johnson syndrome:
- Implicit jaundice.
- A feeling of bitterness in the mouth.
- Pain in the right hypochondrium.
- With Dabin-Johnson syndrome, the liver of patients is enlarged. In the case of Rotor syndrome, it is increased only in some cases.
- Increased bilirubin in the blood.
- Liver function remains normal.
- Blood tests unchanged.
- In liver biopsies, pigment accumulation does not occur.
The prognosis of the disease as a whole is favorable, the syndrome has been going on for many years, not too worsening the human health.
Diagnosis of the syndrome
The disease can only be established with a comprehensive diagnosis of the body.
Studies conducted in the laboratory will help identify elevated levels of bilirubin in the blood. For diagnosis, it is important to know the amount of coproporphyrin in the urine.
In most patients, the disease proceeds without any symptoms.
In the case of diagnosing Rotor syndrome, a liver examination is performed : its x-ray and ultrasound. The gall bladder and ducts undergo the same analyzes.
Disease prevention
General preventive measures are as follows:
- decrease in physical activity;
- treatment of diseases associated with Rotor syndrome;
- stress and emotional stress should be avoided.
Blood bilirubin increase factors
Bilirubin rises for the following reasons:
- severe stress;
- improper diet, violation of diet;
- drug use;
- excessive drinking.
Disease treatment
There are no general methods for eliminating the disease. However, preventive measures must be observed. As medicines, you need to take drugs that remove bilirubin from the body. Often choleretic drugs are used.
The doctor will help you choose the appropriate treatment.