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Vol - 28, Issue - 11
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[This article belongs to Volume - 28, Issue - 11]
International Medical Journal
Journal ID
:
IMJ-28-11-2021-1069
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479
Title
:
A Rare presentation of Acute Severe Jaundice in a young patient
Abstract
: Budd chiari syndrome (BCS) is a rare disorder presenting with cluster of symptoms like pain in abdomen, yellowish discoloration of sclera, abdominal distension, bleeding tendency and sometimes altered sensorium. It is characterised by hepatic venous out flow obstruction at the level of hepatic vein or at the level of inferior vena cava (IVC) (hepatic portion). However, definition of BCS doesn't include the obstruction secondary to cardiac or pericardial disorder or sinusoidal obstruction syndrome. The etiology of BCS may be idiopathic, or secondary to thrombotic disorder or hyper coagulable state or myeloproliferative disorder. Prevalence of BCS is more in Western population as compared to Asians. Patient may be asymptomatic or present with acute fulminant hepatic failure which makes its presentation heterogenous. It may be classified into primary which is mainly due to veno-occlusion / thrombotic process and secondary which peculiarly is due to the external mechanical compression due to malignant tumor. The multi-centric data published, illustrates that around 25-40 % of patients have co-existing prothrombotic condition. Primary BCS, hence is an outcome of prothrombotic etiology. We present here a case of BCS with idiopathic etiology. The patient did not have any prior medical/surgical comorbidity or addiction and belongs to low socio-economic status.
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