Budd-Chiari Syndrome
What is Budd-Chiari Syndrome?
Budd-Chiari syndrome is a rare disorder characterized by the blockage of the veins that transport the blood out from the liver. As a consequence, the blood flow is blocked and the blood accumulates in the liver causing an enlargement of the liver (hepatomegaly). The excess of fluid may leak into the abdominal cavity forming fluid accumulations that cause the abdomen to swell, a condition called ascites.
Symptoms of the syndrome include abdominal pain, jaundice (a yellow coloring of the skin and white part of the eyes) and high blood pressure of the main veins of the liver (portal hypertension).
If left untreated the condition might worsen to cause damage and scarrring (cirrhosis) of the liver and eventually cause life-threatening liver failure. The treatment will vary depending on what is the underlying cause of the vein obstruction. Sometimes an anticoagulant therapy is enough to prevent the formation of blood clots that block the blood flow. Some patients might require a surgical intervention. If cirrhosis has already occurred, a liver transplantation might be needed.
Budd-Chiari syndrome is a rare disorder characterized by the blockage of the veins that transport the blood out from the liver. As a consequence, the blood flow is blocked and the blood accumulates in the liver causing an enlargement of the liver (hepatomegaly). The excess of fluid may leak into the abdominal cavity forming fluid accumulations that cause the abdomen to swell, a condition called ascites.
Symptoms of the syndrome include abdominal pain, jaundice (a yellow coloring of the skin and white part of the eyes) and high blood pressure of the main veins of the liver (portal hypertension).
If left untreated the condition might worsen to cause damage and scarrring (cirrhosis) of the liver and eventually cause life-threatening liver failure. The treatment will vary depending on what is the underlying cause of the vein obstruction. Sometimes an anticoagulant therapy is enough to prevent the formation of blood clots that block the blood flow. Some patients might require a surgical intervention. If cirrhosis has already occurred, a liver transplantation might be needed.
1.5http://www.orpha.net
Last updated 6 Jan 2012, 09:11 PM
6 Jan 2012, 09:11 PM
my son has just had acute buddchiari and been very unwell - no cause yet found, although he does also have renal disease as well - and underwent a successful tipps procedure for this. all ok at the moment although will be on warfarin lifelong i am told...
Last updated 20 May 2009, 11:44 AM
20 May 2009, 11:44 AM
Hi I just want to know if there is not maybe any new treatments out there for budd chiari, or any advice?
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CoRDS, or the Coordination of Rare Diseases at Sanford, is based at Sanford Research in Sioux Falls, South Dakota. It provides researchers with a centralized, international patient registry for all rare diseases. This program allows patients and researchers to connect as easily as possible to help advance treatments and cures for rare diseases. The CoRDS team works with patient advocacy groups, individuals and researchers to help in the advancement of research in over 7,000 rare diseases. The registry is free for patients to enroll and researchers to access.
Enrolling is easy.
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After these steps, the enrollment process is complete. All other questions are voluntary. However, these questions are important to patients and their families to create awareness as well as to researchers to study rare diseases. This is why we ask our participants to update their information annually or anytime changes to their information occur.
Researchers can contact CoRDS to determine if the registry contains participants with the rare disease they are researching. If the researcher determines there is a sufficient number of participants or data on the rare disease of interest within the registry, the researcher can apply for access. Upon approval from the CoRDS Scientific Advisory Board, CoRDS staff will reach out to participants on behalf of the researcher. It is then up to the participant to determine if they would like to join the study.
Visit sanfordresearch.org/CoRDS to enroll.
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Budd chiari treatments
Created by mstadendorff | Last updated 20 May 2009, 11:44 AM
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