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1/21. portal vein thrombosis in paroxysmal nocturnal haemoglobinuria.

    A 28-year-old man was hospitalized with nausea, vomiting, abdominal pain and low-grade fever. He had a 6-month history of paroxysmal nocturnal haemoglobinuria (PNH), and laboratory data showed anaemia and liver dysfunction. An abdominal ultrasonography showed ascites and portal vein thrombosis. After receiving antithrombotic treatment, the portal vein thrombosis did not extend. portal vein thrombosis is very rare but should be considered when we encounter liver dysfunction associated with PNH as well as hepatic vein thrombosis. ultrasonography is very useful in detecting portal vein thrombosis and facilitating early diagnosis. warfarin is very effective in preventing exacerbation of portal vein thrombosis in PNH.
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ranking = 1
keywords = hepatic
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2/21. Fibrinolytic therapy with rt-PA in a patient with paroxysmal nocturnal hemoglobinuria and budd-chiari syndrome.

    Paroxysmal nocturnal hemoglobinuria (PNH) is associated with a high risk of thrombosis, particularly in the peripheral, cerebral, and abdominal veins. We report a patient with an occlusion of the hepatic veins and a slit shape narrowing of the cava inferior consistent with the budd-chiari syndrome in whom intravenous fibrinolytic therapy with recombinant tissue plasminogen activator (rt-PA) was applied. Systemic rt-PA was given in a dose of 25 mg rt-PA over 3 h and 25 mg rt-PA as constant intravenous infusion over the next 21 h leading to an incomplete recanalization. The same protocol was applied again 2 days later, resulting in a complete recanalization of the hepatic veins and the vena cava inferior. Our case shows that exclusive systemic application of rt-PA can result in full anatomic and clinical restoration.
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ranking = 2
keywords = hepatic
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3/21. recurrence of budd-chiari syndrome after liver transplantation in paroxysmal nocturnal hemoglobinuria.

    Venous thrombembolism is a major complication of paroxysmal nocturnal hemoglobinuria (PNH). Often, veins of atypical localization are afflicted, resulting in cerebral, mesenteric, or hepatic venous thrombosis. We present a patient who received an orthotopic liver graft for chronic budd-chiari syndrome in 1988. PNH was the only thrombophilic predisposition identified in this patient. After transplantation, he repeatedly suffered from hemorrhage. Subsequently, the patient discontinued prophylactic anticoagulation nearly 10 years after transplantation. Within 6 months budd-chiari syndrome recurred, but stabilized after anticoagulation therapy with low-molecular-weight heparin was reinstituted. The patient is clinically stable 14 years after receiving the liver graft. Eleven cases of relapsing budd-chiari syndrome have been reported in the literature. Of these, four patients suffered from PNH. All patients transplanted for PNH-associated budd-chiari syndrome in these reports suffered from either major bleeding or thrombosis. In conclusion, patients afflicted with PNH appear to be at high risk of incurring complications after liver transplantation.
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ranking = 1
keywords = hepatic
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4/21. splenectomy for massive splenic infarction unmasks paroxysmal nocturnal hemoglobinuria.

    Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disorder characterized by pancytopenia, hemolysis, and thrombosis. Abdominal vein thrombosis is a life-threatening manifestation of this disease. We present a patient with complete spleen necrosis due to thrombosis of the splenic vessels. After splenectomy, other causes of thrombophilia were excluded and the diagnosis of PNH was established. The patient was put on anticoagulation but despite the prophylactic international normalized ratio maintained over the last 18 months of follow-up, he had another episode of intrahepatic thrombosis which was treated with tissue plasminogen activator thrombolysis.
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ranking = 1
keywords = hepatic
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5/21. Superior sagittal sinus thrombosis and budd-chiari syndrome due to paroxysmal nocturnal hemoglobinuria managed with transjugular intrahepatic portosystemic shunt: a case report.

    BACKGROUND: Paroxysmal nocturnal hemoglobinuria (PNH), caused by somatic mutation of hematopoietic cells, is associated with complement-mediated hemolysis and a hypercoagulable state. Thrombotic complications in this disease are associated with reduced survival. We report a patient with PNH complicated by intracranial venous thrombosis and budd-chiari syndrome, who was managed with transjugular intrahepatic portosystemic shunt. CASE PRESENTATION: A 26-year-old man presented with thrombosis of the superior sagittal and right sigmoid sinuses. Initial investigations did not reveal any underlying cause. Nine months later, he developed hepatic venous thrombosis. At this time, Ham test was positive. flow cytometry confirmed the diagnosis of PNH. The patient was treated with transjugular intrahepatic portosystemic shunt; one episode of stent blockage one month later was managed successfully with balloon dilatation and restenting. CONCLUSION: PNH should be considered in patients with unexplained venous thrombosis. Thrombosis in these patients needs to be managed with prolonged anticoagulation. For budd-chiari syndrome in patients with underlying PNH, transjugular intrahepatic portosystemic shunt may be a good option but caution is needed to prevent stent occlusion.
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ranking = 8
keywords = hepatic
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6/21. Catheter-directed thrombolysis and thrombectomy for the budd-chiari syndrome in paroxysmal nocturnal hemoglobinuria in three patients.

    Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematopoietic stem cell disorder characterized by hemolytic anemia, hemoglobinuria, bone marrow failure, and hypercoagulability. Thrombosis is the leading cause of mortality and occurs in one-half of PNH patients, with the hepatic veins being the most common site. patients with hepatic vein thrombosis (budd-chiari syndrome) can present with abdominal pain, hepatomegaly, jaundice, and ascites. prognosis is poor for these patients; death may occur from liver failure, vessel rupture, intestinal ischemia, infarction, necrosis, or sepsis. The authors report three consecutive cases of successful treatment with catheter-directed thrombolysis and thrombectomy directly in the hepatic veins in patients with PNH who developed acute hepatic vein thrombosis. This treatment represents a potential bridge toward more curative therapies such as allogeneic bone marrow transplant.
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ranking = 4
keywords = hepatic
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7/21. Benign liver-cell adenoma associated with long-term administration of an androgenic-anabolic steroid (methandienone).

    A 19-year-old man with paroxysmal nocturnal hemoglobinuria treated for 3 years with Methandienone was admitted to the hospital with hemoperitoneum due to the rupture of an hepatic tumor. histology revealed that it was a benign liver cell adenoma, with a pathologic appearance and mode of clinical presentation closely resembling those of cases observed to develop in association to contraceptive steroids.
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ranking = 1
keywords = hepatic
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8/21. Paroxysmal nocturnal hemoglobinuria with hepatic vein thrombosis presenting as hepatic encephalopathy.

    We present a patient with paroxysmal nocturnal hemoglobinuria with diffuse hepatic central vein thrombosis who presented with encephalopathy and recovered from the hepatic manifestations with antihepatic coma measures alone.
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ranking = 11
keywords = hepatic
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9/21. A good outcome pregnancy in a patient with paroxysmal nocturnal hemoglobinuria.

    In this report, the maternal and fetal risks in a patient with paroxysmal nocturnal hemoglobinuria (PNH) were evaluated. A patient with PNH carried a pregnancy to successful delivery, with a healthy male infant, and suffered from postpartum cerebral subcortical, but not hepatic, thrombosis followed by recovery. An elevation of serum levels of lactate dehydrogenase isoenzyme, alpha-hydroxybutyrate dehydrogenase, was observed during pregnancy. The level was ameliorated to the non-pregnant level by delivery. However, hemoglobin concentrations during pregnancy and post-partum were at least 8 g/dl. This is a first report that shows tight coupling between an onset of intravenous hemolysis and pregnancy. The management of pregnancies in such patients is also discussed.
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ranking = 1
keywords = hepatic
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10/21. Ultrasonic demonstration of clinical and subclinical hepatic venous thrombosis in paroxysmal nocturnal haemoglobinuria.

    Extensive hepatic venous thrombosis was demonstrated by ultrasonic scanning in three patients with paroxysmal nocturnal haemoglobinuria (PNH). The ultrasonography results were confirmed by X-ray venography. Two of these patients were without clinical symptoms of hepatic involvement apart from a slight increase in transaminases. This study indicates, firstly, that ultrasonic scanning is a rapid and reliable diagnostic tool in monitoring patients for this serious and often fatal complication, and, secondly, that hepatic venous thrombosis may be a more common complication in PNH than previously assumed.
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ranking = 7
keywords = hepatic
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