Diagnosis and management of severe Budd-Chiari syndrome.

Hepatobiliary Pancreat Dis Int

Department of General Surgery, First Affiliated Hospital, Zhengzhou University, Zhengzhou 450052, China.

Published: November 2003

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Budd-Chiari Syndrome as an Initial Manifestation of Incomplete Systemic Lupus Erythematosus.

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Unlabelled: This article describes a case of a 26-year-old female with a history of Evan's syndrome who presented with severe exertional dyspnoea and abdominal discomfort. The patient was diagnosed with chronic Budd-Chiari syndrome, a rare vascular disorder characterized by obstruction of the hepatic vein. We discuss the risk factors, the clinical manifestations, and diagnostic methods for Budd-Chiari syndrome, as well as the possible association with an underlying incomplete systemic lupus erythematosus.

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Behçet's Disease (BD) is a multisystem inflammatory disorder that can lead to severe vascular complications, including Budd-Chiari Syndrome (BCS), a rare but life-threatening condition characterized by hepatic vein obstruction. The co-occurrence of BD and inherited thrombophilia, such as Factor V Leiden mutation, significantly increases the risk of thrombosis, complicating the clinical management of affected individuals. In this case, a 16-year-old female initially presented with nonspecific symptoms of generalized fatigue and bone pain, which later progressed to abdominal distension and significant hepatosplenomegaly.

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Budd-Chiari syndrome (BCS), a rare hepatic vein obstruction condition, poses significant risks during gestation and the postpartum period. We present the case of a 30-year-old primigravida at 32 weeks gestation admitted with weakness and lethargy, which was diagnosed with impending uterine rupture, HELLP syndrome, and intrauterine fetal demise. An emergency cesarean section was performed, delivering a stillborn baby and uncovering 700 milliliters of blood clots in her abdominal cavity.

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