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1/150. Relief of hepatic vein stenosis by balloon angioplasty after living-related donor liver transplantation.

    We have experienced 5 hepatic vein stenoses in 3 children (8 to 23 months old) after living-related liver transplantation (total 48 liver transplants for 48 children between June 1990 and November 1992). The initial symptoms of hepatic vein stenosis were ascites and/or edema. The blood flow of hepatic vessels was monitored by duplex sonography. The mean velocity of the hepatic vein and the portal vein was decreased and flow wave pattern of the stenotic hepatic vein was flat. The patients were treated by percutaneous transhepatic balloon angioplasty. After a successful angioplasty, the mean velocity of the hepatic vein and portal vein increased and pulsatile waves returned to the hepatic vein. Arterial ketone body ratio (acetoacetate/3-hydroxybutylate) increased, promptly followed by recovery of other liver function tests. In 1 patient, this complication occurred three times with intervals of 7 months and 3 months between episodes of hepatic vein stenosis. In conclusion, hepatic vein flow should be monitored routinely with duplex sonography after living-related donor liver transplantation. Percutaneous transhepatic balloon angioplasty is a primary treatment for the stenosis. ( info)

2/150. Defibrotide as salvage therapy for refractory veno-occlusive disease of the liver complicating allogeneic bone marrow transplantation.

    A 30-year-old woman developed veno-occlusive disease of the liver during an allogeneic BMT for acute leukemia. Treatment with recombinant human tissue plasminogen activator and heparin resulted in an incomplete and transient response followed by progressive disease. The patient was then given defibrotide (DF), a mammalian tissue-derived polydeoxyribonucleotide developed for the treatment of a number of vascular disorders, which has thrombolytic and anti-thrombotic properties. No significant bleeding or other major toxicities were observed during treatment and she made a full recovery. At 6 months after the onset of VOD her liver function tests and color flow Doppler ultrasound scan are normal. Our experience supports the preliminary results already obtained with DF. Its efficacy should be evaluated in a prospective randomized fashion. ( info)

3/150. Recurring fibro-obliterative venopathy in liver allografts.

    Recurrent diseases in liver allografts are not uncommon. These occur most frequently in those transplanted for viral hepatitis b and C. We report an unusual case of recurrent process in two consecutive liver allografts received by a 37-year-old woman, who previously had an unremarkable past medical history but developed a rapidly progressive cholestatic liver failure. Histopathologic examination of the native liver showed fibroocclusive lesions of both terminal hepatic venules and portal vein branches. The exuberant fibroobliterative process created dense fibrosis with whorled appearance, and broad fibrous septa connecting adjacent central areas, and sometimes bridging portal to central areas. Dense portal fibrosis resulted in compression atrophy and loss of bile ducts. The first allograft, which failed within 3 months, showed histopathologic findings similar to that of the native liver. A liver biopsy that was performed 20 months after the second liver transplant again showed similar histopathology. The histopathologic features and clinical presentation of this patient suggest an unusual form of recurring progressive fibroobliterative venopathy causing liver failure. ( info)

4/150. Epithelioid hemangioendothelioma with marked liver deformity and secondary budd-chiari syndrome: pathological and radiological correlation.

    A case of malignant epithelioid hemangioendothelioma of the liver in a 48-year-old woman with severe portal hypertension and marked deformity of the liver is presented. This woman had a history of mild liver dysfunction since the age of 30 years, and abdominal distention, esophageal varices, splenomegaly and ascites since October 1996. Imaging examinations revealed liver deformity with severe atrophy of the left lobe and the anterior segment of the right lobe. Celiac arteriography showed narrowing and upward deviation of the proper hepatic artery, and occlusion of the left and right anterior hepatic arteries. Since March 1997, hepatic venography showed stenosis in the right hepatic vein truncus. budd-chiari syndrome was clinically diagnosed. She died in June 1997. The autopsy disclosed massive tumor embolism in the left and right anterior portal branches, few in the hepatic artery, and occlusion of the left and right anterior hepatic arteries. The extensive tumor embolism resulted in portal hypertension, and atrophy of the left lobe. The anterior segment of the right lobe was probably caused by the occlusion of both the hepatic arteries and the portal veins. The posterior segment of the right lobe, without massive tumor embolism in its portal branch, appeared hypertrophic. ( info)

5/150. mycobacterium tuberculosis infection in allogeneic bone marrow transplantation patients.

    Bone marrow transplant (BMT) recipients are prone to bacterial, viral and fungal infections. mycobacterium tuberculosis infection can occur in these patients, but the incidence is lower than that of other infections. This report describes four patients with mycobacterium tuberculosis infection identified from 641 adult patients who received a BMT over a 12-year period (prevalence 0.6%). The pre-transplant diagnosis was AML in two patients and CML in the other two. Pre-transplant conditioning consisted of BU/CY in three patients and CY/TBI in one. Graft-versus-host disease (GVHD) prophylaxis was MTX/CsA in three patients and T cell depletion of the graft in one patient. Sites of infection were lung (two), spine (one) and central nervous system (one). Onset of infection ranged from 120 days to 20 months post BMT. Two patients had co-existing CMV infection. One patient had graft failure. The two patients who received anti-tuberculous (TB) therapy recovered from the infection. Although the incidence of tuberculosis in BMT patients is not as high as in patients with solid organ transplants, late diagnosis due to the slow growth of the bacterium can lead to delay in instituting anti-TB therapy. A high index of suspicion should be maintained, particularly in endemic areas. ( info)

6/150. Late onset veno-occlusive disease following high-dose chemotherapy and stem cell transplantation.

    The original definition of hepatic veno-occlusive disease (VOD), which is still widely accepted, includes onset of the clinical syndrome before day 20 following high-dose chemotherapy (HDC) and stem cell transplantation (SCT). We retrospectively identified four patients following HDC and SCT presenting with late onset VOD occurring at day 24, day 27, day 34 and day 42 post SCT. All patients had moderate VOD, with successful resolution of the VOD before day 100 with optimal supportive therapy. Common risk factors for VOD shared by all four patients included an older age (median age: 60 years), and use of a busulphan-containing regimen. Mean and maximum bilirubin levels for all patients during the VOD syndrome were 2.02, 1.76, 5.09, 2.87 mg/dl and 2.5, 2.2, 8.9 and 4.1 mg/dl, respectively, which correlated well with duration of VOD. All patients encountered platelet transfusion-dependent thrombocytopenia during VOD. ursodeoxycholic acid was used as VOD prophylaxis beginning at a mean of 33 days prior to onset of VOD. As the cellular target of hepatic VOD is as yet unidentified, it is uncertain whether ursodiol or other common characteristics of patients with late onset VOD influence the pathogenesis and natural history of this disease. We believe that the uncommon clinical entity of late onset VOD, a potentially fatal regimen-related toxicity, should not be ignored as a diagnosis of liver disease after 3 or more weeks following HDC and SCT. ( info)

7/150. Blocking of the hepatic vein outflow by neointima covering a wallstent across a membranous stenosis of the inferior vena cava.

    A 31-year-old man presented with idiopathic membranous obstruction of the suprahepatic inferior vena cava (IVC) and was treated by balloon dilation and placement of a Wallstent. The patient improved markedly. However, he developed obstruction of the hepatic vein outflow secondary to neointima formation over the stent that covered the hepatic vein ostia. The patient died of liver failure and septicemia. We believe that this is the first report of such a serious complication. ( info)

8/150. Deep venous thrombosis of the arm after intravenous immunoglobulin infusion: case report and literature review of intravenous immunoglobulin-related thrombotic complications.

    Thrombosis resulting from intravenous immunoglobulin infusion is a relatively unknown complication. We describe a patient who developed deep venous thrombosis of her left arm shortly after intravenous immunoglobulin administration. In addition, we review the thrombotic incidences reported in the literature and the possible association with hepatic veno-occlusive disease after bone marrow transplantation. Measures that can potentially prevent this complication are discussed. ( info)

9/150. Fatal course of veno-occlusive disease of the liver (endophlebitis hepatica obliterans) in a preterm infant.

    We describe the fatal course of a preterm infant of 34 weeks' gestation with veno-occlusive disease of the liver and refractory ascites. Despite aggressive medical management, the baby died twenty-two hours post partum because of cerebral haemorrhage before potentially life-saving organ transplantation could take place. At autopsy, paucity of lymphoid tissue in lymph nodes, thymus, spleen, and gastrointestinal tract were also seen. To our knowledge, this is the youngest infant with veno-occlusive disease of the liver reported in the literature. ( info)

10/150. Antithrombin treatment of severe hepatic veno-occlusive disease in children with cancer.

    hepatic veno-occlusive disease (VOD) is a well-known complication of chemotherapy in wilms tumor patients, particularly young children. Although this complication resolves uneventfully in most patients, fatal cases have been reported. Severe VOD after transplantation has a high mortality rate ranging from 45% to 98%. New hemostatic therapeutic strategies have significantly improved the prognosis of VOD. Chemotherapy-related VOD in wilms tumor usually has a good prognosis. We describe two patients with wilms tumor and one with acute lymphoblastic leukemia, who developed severe veno-occlusive disease of the liver according to the baltimore criteria while undergoing chemotherapy; the symptoms were hepatomegaly, ascites, hyperbilirubinemia, weight gain and, in one patient, short-term lethargy. Elevated LDH levels of 872 to 12,000 U/l were observed in our patients. All patients had thrombocytopenia between 29,000 and 40,000/microl and decreased antithrombin (AT) and protein c levels; two patients had gastrointestinal bleeding. All patients developed a coagulopathy because of severe hepatic dysfunction. Two patients received low-dose heparin at the onset of VOD. The thrombolytic therapy was rapidly changed to AT supplementation (20-80 IU/kg bw 2x per day) without heparin when thrombocytes were very low or gastrointestinal bleeding occurred. Resolution of VOD was observed in all patients receiving AT alone. The chemotherapy was discontinued in a patient with accidental actinomycin D overdosage in view of the severity of symptoms. The remaining two patients received chemotherapy according to the therapy protocol after restitution. All patients survived without sequelae with a median follow-up of 28 months (range 8-48 months). CONCLUSION: hepatic veno-occlusive disease is a rare but increasingly recognized complication in pediatric cancer patients receiving conventional chemotherapy. AT supplementation constitutes a good alternative treatment of severe VOD in comparison with other thrombolytic therapies, particularly in patients at high risk of bleeding. ( info)
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