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11/150. Cavographic study of an early stage of obstruction of the hepatic portion of the inferior vena cava.

    BACKGROUND: Liver disease caused by a chronic lesion of the hepatic portion of the inferior vena cava (IVC) is clinically characterized by dilated superficial veins in the body trunk with cephalad flow, hepatomegaly and splenomegaly. Cavography shows stenosis or complete obstruction near the cava-atrial junction. methods: Early (acute and subacute) forms of the disease were recognized. The early stage of the disease manifested as jaundice, hepatomegaly or ascites and fever. patients with acute and subacute onset of the illness with no past history of liver disease were studied with inferior vena cavography. Some of the patients had repeat cavography at 6 months and at 1 year after the initial investigations. RESULTS: Three types of cavographic lesions were observed in the early stages of the disease: type 1, linear lucent area in the IVC close to cava-atrial junction; type 2, a smooth or irregular narrowing of almost the whole segment of the hepatic portion of the IVC; and type 3, a constriction or narrowing of a segment of the IVC near the cava-atrial junction. The first two types were associated with the acute stage of the disease and type 3 with the subacute stage. Type 2 and 3 lesions were associated with post-stenotic dilatation (PSD) close to the atrium. Lucent areas resulting from thrombosis are common in PSD. The acute and subacute hepatic IVC diseases in nepal are commonly associated with bacterial infection. CONCLUSIONS: It is postulated that the early cavographic lesions are consistent with thrombosis and thrombophlebitis of the hepatic portion of the IVC, and that resolution of the lesions leads to the development of stenosis and to complete obstruction. ( info)

12/150. Acute on chronic phenomenon in hepatic IVC obstruction: a case report.

    Hepatic IVC obstruction though common is usually misdiagnosed because of lack of appreciation of the disease. Patient with chronic disease may develop acute exacerbation, which may be precipitated by surgery or endoscopic procedures. It is a report of a case of chronic IVC disease with acute development of ascites following gallbladder surgery. ( info)

13/150. Gastric varices with splenic vein occlusion treated by splenic arterial embolization.

    A 53-year-old man was admitted to our hospital in August 1997 with enlarged gastric varices. Computed tomography (CT) showed splenic vein occlusion, gastric varices, and extra-gastric wall collateral veins. color flow images of gastric varices were clearly visualized, and the velocity in the gastric varices was 19.6 cm/s via endoscopic color Doppler ultrasonography (ECDUS). The patient was diagnosed with gastric varices according to angiographic findings of splenic vein occlusion, and splenic arterial embolization was performed. Two weeks after the splenic arterial embolization, CT showed peripheral areas of low attenuation in the spleen, due to splenic infarction, with 70% of the spleen volume showing low attenuation. Eight months after the splenic arterial embolization, ECDUS revealed a decrease in gastric variceal color flow images, with the velocity in the gastric varices being 10.3 cm/s. ( info)

14/150. N-acetylcysteine for hepatic veno-occlusive disease after allogeneic stem cell transplantation.

    Three patients developed veno-occlusive disease of the liver (VOD) after allogeneic stem cell transplantation. On the day after diagnosis, N-acetylcysteine (NAC) was given, initially in loading doses and thereafter 50-150 mg/kg/day for 12 to 31 days. The maximum bilirubin levels were 137, 58 and 138 mmol/l in the three patients, respectively. After the introduction of NAC, bilirubin, aspartate aminotransferase, sIL-2 receptor and IL-8 decreased. All three patients achieved normal bilirubin levels and prothrombin times. To conclude, NAC may be useful for treatment of VOD. ( info)

15/150. MR imaging findings in two patients with hepatic veno-occlusive disease following bone marrow transplantation.

    The aim of this study was to describe the MRI features of veno-occlusive disease (VOD) following bone-marrow transplantation in two patients. The MRI features consisted of hepatomegaly, hepatic vein narrowing, periportal cuffing, gallbladder wall thickening, marked hyperintensity of the gallbladder wall on T2-weighted images, ascites, and pleural effusion. In one patient, signs of reduced portal venous flow velocity were also observed. It is concluded that the use of MRI as a complementary technique following non-conclusive US examination enabled a timely diagnosis of this life-threatening disease in both patients. ( info)

16/150. Application of nitric oxide for a case of veno-occlusive disease after peripheral blood stem cell transplantation.

    A 5-year-old girl at high risk for acute lymphoblastic leukemia was treated with high-dose chemotherapy and autologous peripheral blood stem cell transplantation (PBSCT). However, her condition was complicated by veno-occlusive disease of the liver (VOD) after PBSCT. For treatment of VOD, transdermal isosorbide tape was applied as a nitric oxide (NO) donor. The signs of VOD improved immediately after NO treatment was initiated, and the patient showed no side effects from the transdermal isosorbide tape. ( info)

17/150. Management of hepatic venous obstruction after split-liver transplantation.

    Stenosis of the hepatic vein anastomosis is an unusual but critical complication after liver transplantation. In pediatric liver transplantation, the scarcity of size-matched donors has required the use of segmental liver allografts, either as reduced-size or split-liver grafts. This report illustrates the primary use of a hepatic vein stent to manage hepatic venous outflow obstruction in a pediatric split-liver recipient, and reviews experience in the management of hepatic venous outflow obstruction after liver transplant using stent methods. ( info)

18/150. Focal elastic obstruction of the inferior vena cava.

    Obstruction of the supra-hepatic inferior vena cava (IVC) is a common cause of hepatic venous hypertension and the most common cause of budd-chiari syndrome. Because most cases of IVC obstruction go undiagnosed until budd-chiari syndrome develops, the natural history of IVC obstruction is not well defined. We report a case of a focal, elastic, non-membranous obstruction of the IVC causing hepatic venous hypertension and elevated serum transaminases in a 36-year-old man. The obstruction was successfully treated with placement of a self-expanding metallic stent with normalization of hepatic transaminases. ( info)

19/150. Successful therapy of transplant-associated veno-occlusive disease with a combination of tissue plasminogen activator and defibrotide.

    A 36-year-old man underwent matched unrelated donor bone marrow transplantation for chronic myeloid leukaemia. He developed severe hepatic veno-occlusive disease as an early post-transplant complication. tissue plasminogen activator was initially felt to be contraindicated since the patient had concomitant pericarditis. Defibrotide was therefore commenced as treatment for veno-occlusive disease. The pericarditis improved but the veno-occlusive disease continued to worsen (peak bilirubin 353 micromol/l). tissue plasminogen activator followed by a heparin infusion was therefore administered. However, he proceeded to develop haemorrhagic cardiac tamponade that required drainage. Thrombolysis was therefore discontinued and treatment with defibrotide resumed after an interval of 48 h. The veno-occlusive disease gradually resolved and defibrotide was discontinued once the bilirubin had plateaued. He was discharged home on day 52 post-transplant. ( info)

20/150. hepatic veno-occlusive disease in two patients with relapsed acute myeloid leukemia treated with anti-CD33 calicheamicin (CMA-676) immunoconjugate.

    Monoclonal antibodies recognizing hematopoietic antigens are increasingly being used to target therapy directly at leukemic cells, with the aim of achieving sustained remission with little systemic toxicity. Administration of anti-CD33 calicheamicin immunoconjugate is commonly regarded as being safe, with only moderate systemic non-hematological side effects. We report on two cases of hepatic veno-occlusive disease in heavily pretreated patients presenting with relapsed acute myeloid leukemia (AML). Since significant liver toxicity prevented further specific therapy in both patients, we recommend that antibody therapy with anti-CD33 immunoconjugate should be applied with caution in patients presenting with risk factors for the development of hepatic veno-occlusive disease. ( info)
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