Figure 1 - uploaded by Amey Dilip Sonavane
Content may be subject to copyright.
& 2: MRI DYNAMIC LIVER (Axial viewvenous phase) revealing hepatomegaly with nodular margins and significantly enlarged caudate lobe. Discrete hepatic veins are not visualized. Moderate ascites is also noted.
Source publication
A serious complication of Paroxysmal Nocturnal Hemoglobinuria (PNH) is hepatic venous thrombosis leading to Budd-Chiari Syndrome (BCS). Mortality due to untreated BCS is high despite aggressive treatment. Liver Transplantation in PNH is riddled with the risk of intraoperative and postoperative thrombotic complications, which can cause recurrent BCS...
Similar publications
A Política Nacional de Humanização do SUS tem na diretriz do acolhimento uma estratégia de humanização dos serviços de saúde em qualquer nível de atenção. O acolhimento do usuário deve fazer parte de toda linha de cuidado na Rede de Assistência em Saúde (RAS), de modo a garantir o atendimento de forma continuada, integral e resolutiva. O objetivo d...
Citations
... [2] A serious complication of Paroxysmal Nocturnal Hemoglobinuria (PNH) is hepatic venous thrombosis leading to Budd-Chiari Syndrome (BCS). [3]. Obstruction of the hepatic venous outflow tract, either at the level of hepatic veins or the inferior vena cava, is referred to as the Budd Chiari Syndrome. ...
PNH is acquired disorder of hematopoietic stem cell which is characterized by acquired intravascular hemolytic anemia, bone marrow failure with pancytopenia and high incidence of life threatening thrombosis. A serious complication of PNH is hepatic venous thrombosis leading to Budd Chiari syndrome. Budd-Chiari syndrome (BCS) is an uncommon disorder characterized by obstruction of hepatic venous outflow. The obstruction may be thrombotic or non-thrombotic anywhere along the venous course from the hepatic venules to junction of the inferior vena cava (IVC) to the right atrium. Budd-Chiari syndrome is a congestive hepatopathy caused by blockage of hepatic veins. This syndrome occurs in 1/100 000 in the general population. We present a rare case of PNH in a 23 year old man clinically presenting with Budd Chiari syndrome that on detailed investigations actually turned out to be complication of PNH. This article emphasizes on early detection of PNH to prevent life threatening complications like Budd Chiari syndrome.
