2024-04-18 06:26:48
Budd-Chiari syndrome can be dangerous if left untreated. What are the symptoms of liver disease and what does it mean for life expectancy.
Budd-Chiari syndrome is a rare liver disease in which blood accumulates in the liver because the hepatic veins or the inferior vena cava are blocked.
Normally, blood from the liver flows through several veins into the vena cava, which then carries it to the heart. However, if one or more of these veins are blocked, for example by a blood clot, blood flow is restricted. Then more and more blood collects in the liver, which can cause numerous problems. In the worst case, they can reach life-threatening proportions. To prevent this, prompt treatment is important.
What are the symptoms of Budd-Chiari syndrome?
Those who suffer from Budd-Chiari syndrome do not always notice it right away – some affected people have no or only mild symptoms. But they are in the minority.
Usually, Budd-Chiari syndrome causes obvious and sometimes stressful symptoms, mainly the following:
- stomach pain
- significant enlargement of the liver
- increased abdominal circumference
The liver swells because more and more blood is building up in it. This means that the organ can also be sensitive to pressure.
Abdominal circumference increases because fluid collects in the abdominal cavity. This in turn is the result of blood congestion in the liver: the pressure in the blood vessels of the liver becomes so high that fluid escapes from them into the abdominal cavity.
In addition to these symptoms typical of Budd-Chiari syndrome, other symptoms sometimes occur. These include, for example:
- Nausea, feeling of fullness and loss of appetite
- Fatigue and reduced resilience
- Yellowing of the skin (jaundice)
- bloody vomiting due to bleeding from varicose veins in the esophagus
However, none of the symptoms mentioned are specific to Budd-Chiari syndrome. So they can also have other causes – this is quite likely in Germany: Budd-Chiari syndrome is a rare disease in this country.
Incidence of Budd-Chiari syndrome
Budd-Chiari syndrome is also rare in other Western countries. It is estimated that around one in 2.5 million people in the Western population develop Budd-Chiari syndrome each year. However, the syndrome occurs more often in countries with worse living conditions.
In principle, the disease can start at any age. However, adults between the ages of 30 and 50 are most often affected.
Who is at risk of developing Budd-Chiari syndrome?
In many cases, Budd-Chiari syndrome is caused by blood clots that block the veins of the liver. This can occur, among other things, in various diseases where the blood clots more easily than it should, or which affect the condition of the blood in other ways.
One of the following diseases or effects is often identified in people with Budd-Chiari syndrome:
- genetically determined malignant diseases in which the blood-forming cells in the bone marrow multiply abnormally (myeloproliferative neoplasms)
- Hereditary coagulation disorder (eg due to congenital deficiency of protein C, protein S, antithrombin III or factor V Leiden mutation)
- Taking birth control pills
- pregnancy
- chronic inflammatory bowel disease
Less commonly, the syndrome occurs when one or more liver veins are narrowed, which means that the blood flow is impaired from the outside – for example by a tumor that puts pressure on the vein.
What helps against Budd-Chiari syndrome?
If caught early, Budd-Chiari syndrome is usually easy to treat. Therapy often involves several measures. Among other things, medicines intended to dissolve blood clots, so-called anticoagulants, are used. If these are not enough, there are other therapeutic options to restore blood flow. One option, for example, is a surgical procedure in which an artificial connection (so-called shunt) is created between the portal vein and the hepatic vein.
How does Budd-Chiari syndrome affect life expectancy?
Without treatment, Budd-Chiari syndrome usually leads to death within a few months to three years. Patients who seek treatment in time have a much longer life expectancy: around 90 out of every 100 affected people are still alive five years after diagnosis.
In principle, however, life expectancy also depends on which disease caused the blockage of the hepatic vein and how well it can be brought under control.
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