The key facts
- The Health Ministry included primary biliary cholangitis in the list of rare (orphan) diseases
- The list of orphan diseases now contains 306 entries
- The disease can progress for a long time without pronounced symptoms
The Disease Recognized as Rare
Russia's Ministry of Health has included primary biliary cholangitis in the list of rare, or orphan, diseases. The updated version of the document has been published on the ministry's official website. It is a chronic autoimmune liver disease that was previously also called primary biliary cirrhosis. With the addition of the new disease, the Russian list of orphan diseases now contains 306 entries.
The Nature of the Disease
In primary biliary cholangitis, the immune system mistakenly attacks the small bile ducts inside the liver. The gradual destruction of the ducts disrupts the flow of bile, which can lead to damage to liver tissue. As the disease progresses, the risk of developing fibrosis and liver cirrhosis increases.
Symptoms
Among the characteristic symptoms, doctors identify constant fatigue, weakness, and itchy skin. Some patients also experience jaundice, pain, or discomfort in the right upper abdomen. At the same time, the disease can remain without pronounced manifestations for a long time.
Other Health Ministry Updates
Earlier, Russia's Ministry of Health updated the standard of medical care for adult patients with recurrent depressive disorder, expanding the list of diagnostic and treatment methods. The standard includes electroconvulsive therapy, transcranial magnetic stimulation, brain electroneurostimulation, and other procedures, as well as the possibility of using plasmapheresis, blood ultrafiltration, and physiotherapy methods. The list of examinations was supplemented with tests for HIV, hepatitis B and C, and antibodies to pale treponema. The document also provides for consultations with individual specialists, including a urologist, and dispensary observation by a psychiatrist.
