Budd-Chiari syndrome natural history, complications and prognosis: Difference between revisions
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{{Budd-Chiari syndrome}} | {{Budd-Chiari syndrome}} | ||
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==Overview== | ==Overview== | ||
If left untreated, patients with Budd-Chiari syndrome have a high mortality rate.About 90% die within 3 years as the disease may | The [[symptoms]] of Budd-Chiari syndrome usually develop in the third or fourth decade of life and start with [[symptoms]] such as [[hepatomegaly]], [[ascites]], and [[abdominal pain]]. Without treatment, the patient will develop [[complications]] such as [[hepatic encephalopathy]], [[Variceal bleeding|variceal hemorrhage]], [[hepatorenal syndrome]], [[portal hypertension]], [[complications]] secondary to [[Decompensation|hepatic decompensation]], which may eventually lead to intractable [[ascites]] with [[emaciation]], [[gastrointestinal bleeding]], and [[liver failure]]. [[Complications]] that can develop as a result of Budd-Chiari syndrome are [[portal hypertension]], [[hepatic encephalopathy]], [[Variceal bleeding|variceal hemorrhage]], [[hepatorenal syndrome]], [[Decompensation|hepatic decompensation]], [[bacterial peritonitis]], especially following [[paracentesis]]. If left untreated, patients with Budd-Chiari syndrome have a [[Mortality rate|high mortality rate]]. About 90% die within 3 years as the disease may progress to develop intractable [[ascites]] with [[emaciation]], [[gastrointestinal bleeding]] and [[liver failure]]. [[Prognosis]] is generally good with treatment, and the 5-year [[survival rate]] of patients with treatment of Budd-Chiari syndrome is approximately 74 %. Poor [[Prognostic|prognostic factors]] include older age at [[diagnosis]], [[chronic disease]], [[Hepatic failure|severe liver failure]] and associated refractory [[ascites]]. | ||
==Natural History== | ==Natural History== | ||
*The [[symptoms]] of Budd-Chiari syndrome usually develop in the third or fourth decade of life and start with [[symptoms]] such as [[hepatomegaly]], [[ascites]], and [[abdominal pain]].<ref name="pmid28922103">{{cite journal |vauthors=Grus T, Lambert L, Grusová G, Banerjee R, Burgetová A |title=Budd-Chiari Syndrome |journal=Prague Med Rep |volume=118 |issue=2-3 |pages=69–80 |year=2017 |pmid=28922103 |doi=10.14712/23362936.2017.6 |url=}}</ref> | |||
*Without treatment, the patient will develop [[complications]] like [[hepatic encephalopathy]], [[Variceal bleeding|variceal hemorrhage]], [[hepatorenal syndrome]], [[portal hypertension]], Complications secondary to [[Decompensation|hepatic decompensation]], which may eventually lead to [[Ascites|intractable ascites]] with [[emaciation]], [[gastrointestinal bleeding]], and [[liver failure]]. | |||
==Complications== | ==Complications== | ||
*[[Complications]] that can develop as a result of Budd-Chiari syndrome are:<ref name="pmid28922103">{{cite journal |vauthors=Grus T, Lambert L, Grusová G, Banerjee R, Burgetová A |title=Budd-Chiari Syndrome |journal=Prague Med Rep |volume=118 |issue=2-3 |pages=69–80 |year=2017 |pmid=28922103 |doi=10.14712/23362936.2017.6 |url=}}</ref><ref name="pmid28834866">{{cite journal |vauthors=Lin M, Zhang F, Wang Y, Zhang B, Zhang W, Zou X, Zhang M, Zhuge Y |title=Liver cirrhosis caused by chronic Budd-Chiari syndrome |journal=Medicine (Baltimore) |volume=96 |issue=34 |pages=e7425 |year=2017 |pmid=28834866 |pmc=5571988 |doi=10.1097/MD.0000000000007425 |url=}}</ref> | |||
**[[Portal hypertension]] | |||
**[[Hepatic encephalopathy]] | |||
**[[Variceal bleeding|Variceal hemorrhage]] | |||
**[[Hepatorenal syndrome]] | |||
**[[Decompensation|Hepatic decompensation]] | |||
**[[Bacterial peritonitis]] especially the following [[paracentesis]] | |||
==Prognosis== | ==Prognosis== | ||
*The [[prognosis]] of Budd-Chiari syndrome (BCS) is good with treatment.<ref name="pmid16174064">{{cite journal |vauthors=Khuroo MS, Al-Suhabani H, Al-Sebayel M, Al Ashgar H, Dahab S, Khan MQ, Khalaf HA |title=Budd-Chiari syndrome: long-term effect on outcome with transjugular intrahepatic portosystemic shunt |journal=J. Gastroenterol. Hepatol. |volume=20 |issue=10 |pages=1494–502 |year=2005 |pmid=16174064 |doi=10.1111/j.1440-1746.2005.03878.x |url=}}</ref><ref name="pmid25754880">{{cite journal |vauthors=Qi X, Ren W, Wang Y, Guo X, Fan D |title=Survival and prognostic indicators of Budd-Chiari syndrome: a systematic review of 79 studies |journal=Expert Rev Gastroenterol Hepatol |volume=9 |issue=6 |pages=865–75 |year=2015 |pmid=25754880 |doi=10.1586/17474124.2015.1024224 |url=}}</ref><ref name="pmid19723029">{{cite journal |vauthors=Montano-Loza AJ, Tandon P, Kneteman N, Bailey R, Bain VG |title=Rotterdam score predicts early mortality in Budd-Chiari syndrome, and surgical shunting prolongs transplant-free survival |journal=Aliment. Pharmacol. Ther. |volume=30 |issue=10 |pages=1060–9 |year=2009 |pmid=19723029 |doi=10.1111/j.1365-2036.2009.04134.x |url=}}</ref> | |||
*Without treatment, Budd-Chiari syndrome will result in death as a result of [[Liver failure|progressive liver failure]] within 3 years from [[diagnosis]]. | |||
*Budd-Chiari syndrome is associated with a 10-year [[Survival rates|survival rate]] of 55% with treatment. Among patientd with [[liver transplantation]], the 5-year [[Survival rates|survival rate]] is 70%. | |||
*In [[patients]] treated by [[endovascular]] recanalization the [[Survival rates|5-year survival]] is 86% in patients with intermediate disease severity and 77% in [[patients]] with severe BCS. | |||
*[[Transjugular intrahepatic portosystemic shunt|Transjugular intrahepatic portosystemic shunt (TIPS)]] has a [[Survival rates|5-year survival]] of 78% without the need for [[liver transplantation]]. | |||
*Calculation of [[Prognosis|prognostic index]] for Budd-Chiari can be done using the following formula: | |||
*Prognostic index = (ascites score x 0.75) + (Pugh score x 0.28) + (age x 0.037) + ([[creatinine]] level x 0.0036). | |||
*Prognostic index score less than 5.4 is associated with a [[Prognosis|good prognosis.]] | |||
=== Factors associated with a good prognosis === | |||
Good [[Prognostic|prognostic factors]] include: | |||
*Younger age at [[diagnosis]] | |||
*Low [[Child-Pugh score]] | |||
*Absence of [[ascites]] or easily controlled [[ascites]] | |||
*Low serum [[creatinine]] level | |||
=== Factors associated with a poor prognosis === | |||
Poor [[Prognostic|prognostic factors]] include: | |||
*Older age at [[diagnosis]] | |||
*[[Chronic disease]] | |||
*[[Liver failure|Severe liver failure]] | |||
*[[Ascites|Refractory ascites]] | |||
==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
Latest revision as of 19:24, 30 November 2017
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mazia Fatima, MBBS [2]
Overview
The symptoms of Budd-Chiari syndrome usually develop in the third or fourth decade of life and start with symptoms such as hepatomegaly, ascites, and abdominal pain. Without treatment, the patient will develop complications such as hepatic encephalopathy, variceal hemorrhage, hepatorenal syndrome, portal hypertension, complications secondary to hepatic decompensation, which may eventually lead to intractable ascites with emaciation, gastrointestinal bleeding, and liver failure. Complications that can develop as a result of Budd-Chiari syndrome are portal hypertension, hepatic encephalopathy, variceal hemorrhage, hepatorenal syndrome, hepatic decompensation, bacterial peritonitis, especially following paracentesis. If left untreated, patients with Budd-Chiari syndrome have a high mortality rate. About 90% die within 3 years as the disease may progress to develop intractable ascites with emaciation, gastrointestinal bleeding and liver failure. Prognosis is generally good with treatment, and the 5-year survival rate of patients with treatment of Budd-Chiari syndrome is approximately 74 %. Poor prognostic factors include older age at diagnosis, chronic disease, severe liver failure and associated refractory ascites.
Natural History
- The symptoms of Budd-Chiari syndrome usually develop in the third or fourth decade of life and start with symptoms such as hepatomegaly, ascites, and abdominal pain.[1]
- Without treatment, the patient will develop complications like hepatic encephalopathy, variceal hemorrhage, hepatorenal syndrome, portal hypertension, Complications secondary to hepatic decompensation, which may eventually lead to intractable ascites with emaciation, gastrointestinal bleeding, and liver failure.
Complications
- Complications that can develop as a result of Budd-Chiari syndrome are:[1][2]
Prognosis
- The prognosis of Budd-Chiari syndrome (BCS) is good with treatment.[3][4][5]
- Without treatment, Budd-Chiari syndrome will result in death as a result of progressive liver failure within 3 years from diagnosis.
- Budd-Chiari syndrome is associated with a 10-year survival rate of 55% with treatment. Among patientd with liver transplantation, the 5-year survival rate is 70%.
- In patients treated by endovascular recanalization the 5-year survival is 86% in patients with intermediate disease severity and 77% in patients with severe BCS.
- Transjugular intrahepatic portosystemic shunt (TIPS) has a 5-year survival of 78% without the need for liver transplantation.
- Calculation of prognostic index for Budd-Chiari can be done using the following formula:
- Prognostic index = (ascites score x 0.75) + (Pugh score x 0.28) + (age x 0.037) + (creatinine level x 0.0036).
- Prognostic index score less than 5.4 is associated with a good prognosis.
Factors associated with a good prognosis
Good prognostic factors include:
- Younger age at diagnosis
- Low Child-Pugh score
- Absence of ascites or easily controlled ascites
- Low serum creatinine level
Factors associated with a poor prognosis
Poor prognostic factors include:
- Older age at diagnosis
- Chronic disease
- Severe liver failure
- Refractory ascites
References
- ↑ 1.0 1.1 Grus T, Lambert L, Grusová G, Banerjee R, Burgetová A (2017). "Budd-Chiari Syndrome". Prague Med Rep. 118 (2–3): 69–80. doi:10.14712/23362936.2017.6. PMID 28922103.
- ↑ Lin M, Zhang F, Wang Y, Zhang B, Zhang W, Zou X, Zhang M, Zhuge Y (2017). "Liver cirrhosis caused by chronic Budd-Chiari syndrome". Medicine (Baltimore). 96 (34): e7425. doi:10.1097/MD.0000000000007425. PMC 5571988. PMID 28834866.
- ↑ Khuroo MS, Al-Suhabani H, Al-Sebayel M, Al Ashgar H, Dahab S, Khan MQ, Khalaf HA (2005). "Budd-Chiari syndrome: long-term effect on outcome with transjugular intrahepatic portosystemic shunt". J. Gastroenterol. Hepatol. 20 (10): 1494–502. doi:10.1111/j.1440-1746.2005.03878.x. PMID 16174064.
- ↑ Qi X, Ren W, Wang Y, Guo X, Fan D (2015). "Survival and prognostic indicators of Budd-Chiari syndrome: a systematic review of 79 studies". Expert Rev Gastroenterol Hepatol. 9 (6): 865–75. doi:10.1586/17474124.2015.1024224. PMID 25754880.
- ↑ Montano-Loza AJ, Tandon P, Kneteman N, Bailey R, Bain VG (2009). "Rotterdam score predicts early mortality in Budd-Chiari syndrome, and surgical shunting prolongs transplant-free survival". Aliment. Pharmacol. Ther. 30 (10): 1060–9. doi:10.1111/j.1365-2036.2009.04134.x. PMID 19723029.