True about Budd-chiari syndrome -a) Associated with coagulopathyb) Cirrhosis may occur c) Good prognosisd) Ascites may be present e) Hepatic vein thrombosis
Correct Answer: abde
Description: Budd Chiari syndrome may be predisposed by hypercoagulable conditions, eg deficiency of antithrombin, protein C & S.
Clinical features
The acute syndrome presents with rapidly progressive - severe upper abdominal pain, jaundice, hepatomegaly (enlarge liver), ascites, elevated liver enzymes and eventual encephalopathy.
The fulminant syndrome presents early with encephalopathy and ascites. Severe hepatic necrosis and lactic acidosis may be present as well. Caudate lobe hypertrophy is often present. The majority of patients have a slower-onset form of Budd-chiari syndrome. A system of venous collaterals may form around the occlusion which may be seen on imaging as a "spider's web". Patients may progress to cirrhosis and show signs of liver failure.
An asymptomatic form may be totally silent and discovered only incidently. It is generally not concerning.
Prognosis
Several studies have attempted to predict the survival of patients with Budd-chiari syndrome.
In general, nearly 2/3 of patients with Budd- chiari are alive at 10 years.
Important negative prognostic indicators include ascites, encephalopathy, elevated Child-Pugh scores, elevated prothrombin time and altered serum levels of various substance(sodium, creatinine, albumin and bilirubin).
Survival is also highly dependent on the underlying cause of the Budd-chiari syndrome, eg patients with myeloproliferative disorders may progress to acute leukemia independent of Budd-chiari syndrome.
Category:
Pathology
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