1/162. Successful recanalization of late portal vein thrombosis after liver transplantation using systemic low-dose recombinant tissue plasminogen activator.portal vein thrombosis (PVT) is an infrequent complication following hepatic transplantation. However, deterioration of liver function and accompanying complications may be life threatening. Several attempts of surgical or percutaneous transhepatic procedures have been described. In some cases high dose fibrinolytic regimens have been successful. We describe the case of a male liver recipient with recurrent liver fibrosis due to hepatitis b reinfection and late portal vein thrombosis 45 months after transplantation. Complete recanalization was achieved using systemic low dose recombinant tissue plasminogen activator (rt-PA).- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
2/162. Orthotopic liver transplantation in a patient with severe haemophilia A and with advanced liver cirrhosis.A patient with severe haemophilia A underwent orthotopic liver transplantation because of changes correlated to end-stage liver cirrhosis due to hepatitis b, C and D infection. Replacement therapy was carried out for 4 days and the clinical course was uneventful. At the time of reporting the patient has a normal working life. FVIII plasma concentration is normal. The indirect hyperbilirubinaemia may be related to the Gilbert's anomaly of the donor.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
3/162. Correction of both prothrombin time and primary haemostasis by recombinant factor vii during therapeutic alcohol injection of hepatocellular cancer in liver cirrhosis.We evaluated the efficacy of recombinant factor vii to correct impaired haemostasis in a patient with liver cirrhosis requiring an invasive procedure. A test intravenous bolus of 80 microg/kg of recombinant factor vii was given to a Jehovah's Witness, with a solitary 4.4-cm hepatocellular carcinoma and underlying hepatitis c virus cirrhosis, in an attempt to correct his haemostatic disorders and safely inject the tumour with alcohol. An extensive portal block had precluded consideration of liver transplantation. Haemostasis was evaluated by clotting assays, bleeding time and thromboelastography 10 min before and 10 min and 1, 2, 4, 8 and 24 h after factor vii infusion. Parameters of both coagulation (prothrombin time) and platelet function (bleeding time and the alpha and ma parameters of thrombelastography) were improved 10 min after factor vii infusion; improvements lasted 4 to 8 h or more. platelet count did not change and there was no evidence of disseminated intravascular coagulation. The improvements in haemostatic parameters correlated significantly with the increases in factor vii plasma concentrations (p<0.04). factor vii clearance was 25.1 U/h/kg and its half-life was 5.8 h. The same dose of recombinant factor vii was given to the patient 1 week later, just before the alcohol injections. The patient had no subsequent bleeding or other complication, with no change in haemoglobin levels over 24 h. Thus, recombinant factor vii represents a therapeutic advance, as it can correct fully both coagulation and platelet function defects in cirrhosis and allow invasive procedures to be performed safely.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
4/162. Syndrome of autosomal recessive polycystic kidneys with skeletal and facial anomalies is not linked to the ARPKD gene locus on chromosome 6p.We report on two sibs, both males, one born at 37 the other at 24 weeks of gestation, both with a syndrome similar to that seen in three sets of sibs by Gillessen-Kaesbach et al. [1993: Am J Med Genet 45:511-518]. Both propositi had polycystic kidneys and hepatic fibrosis indistinguishable from that seen in autosomal recessive polycystic kidney disease (ARPKD), and skeletal and facial anomalies. Skeletal abnormalities included "butterfly" vertebrae, square shape of pelvis, and brachymelia. The facial anomalies included hypertelorism, epicanthic folds, and anteverted nares. Additional external findings were apparently low-set ears and a short neck. Histopathological examination of the kidneys showed radial orientation and cystic dilatation of the cortical and medullar tubules. The liver showed "congenital hepatic fibrosis." The hepatic findings in the second infant were less severe. Renal abnormalities were limited to focal tubular cystic changes. Linkage analysis with polymorphic markers of the region 6p21.1-p12, flanking the gene locus of ARPKD, showed different haplotypes in the sibs, thus excluding the ARPKD gene locus in this family and indicating genetic heterogeneity.- - - - - - - - - - ranking = 62.439947850447keywords = family (Clic here for more details about this article) |
5/162. Long-chain 3-hydroxyacyl-coa dehydrogenase deficiency and early-onset liver cirrhosis in two siblings.We present the clinical, pathological, biochemical, and molecular results on an infant girl with long-chain 3-hydroxyacyl-coenzyme a dehydrogenase deficiency and data on her deceased elder brother for whom this condition was retrospectively diagnosed. Clinical signs were liver enlargement and elevated liver enzymes, failure to thrive, and neurological disease (coma, seizures) triggered by an infectious stress. In the second child hepatic failure and status epilepticus developed during the onset of a rotavirus gastroenteritis. A barbituric coma was induced, but hypotonia and lack of eye pursuit persisted after suppression of antiepileptic drugs. She ultimately died of heart failure. Unlike previously reported cases, both of these patients had early-onset cirrhosis, and severe neurological disease was observed in the second child. CONCLUSION: liver cirrhosis and brain damage may be underestimated in cases of long-chain 3-hydroxyacyl-coenzyme a dehydrogenase deficiency and may occur early in life.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
6/162. Bell's palsy during interferon therapy for chronic hepatitis c infection in patients with haemorrhagic disorders.Two adult patients with life-long severe haemorrhagic disorders commenced on interferon-alpha2b therapy for chronic hepatitis c infection. Both developed Bell's palsy several weeks after commencing therapy, They were started on steroids and, in addition, the first patient discontinued interferon-alpha2b therapy while the second patient elected to continue with therapy. In both cases facial paralysis improved over the ensuing weeks. Bell's palsy is often idiopathic but has been reported. in association with herpesviruses. It is not a recognised complication of chronic hepatitis b or C infection, or interferon-alpha2b therapy. However, the interferons are associated with numerous adverse reactions including various neuropsychiatric manifestations and neurological syndromes. There are several reports of nerve palsies, including optic tract neuropathy, occurring during interferon therapy, and immune-based mechanisms are thought to play a role in the aetiopathogenesis. No reports of Bell's palsy in association with interferon therapy were identified in our literature search, although one possible case has been reported to the Committee of safety in medicine. Although Bell's palsy in our patients may have occurred by chance, a neuropathic effect of interferon-alpha2b on the facial nerve cannot be excluded and we urge physicians using interferons to be aware of this potential side-effect.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
7/162. Multiple macroregenerative nodules in liver cirrhosis due to budd-chiari syndrome. case reports and review of the literature.Clinical, radiological, histological and immunohistochemical methods were used to define the nature of multiple rapidly growing hepatic nodules in 2 young patients with budd-chiari syndrome due to myeloproliferative diseases. In one patient, the arterial hyperperfusion of large nodules was demonstrated by dynamic computed tomography and angiography. The explanted livers of these patients showed multiple well-demarcated nodules up to 3 cm in diameter on the background of liver cirrhosis resulting from chronic hepatic congestion. Histologically, these nodules covered a spectrum ranging from adenoma-like lesions to nodules resembling focal nodular hyperplasia. They consisted of essentially normal hepatocytes, and variably contained fibrous septa including neoductules and large, mostly dysmorphic arteries. Sometimes, they were located close to still patent or recanalized veins. These rapidly growing hepatic nodules are best defined as macroregenerative nodules. The knowledge of this entity may help the physician to avoid misinterpretation of such nodules as carcinomas.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
8/162. Childhood cirrhosis associated with alpha-1-antitrypsin deficiency. A genetic, biochemical, and morphologic study.A study of 27 relatives of a child with fatal hepatic cirrhosis due to homozygous Z variant alpha-1-antitrypsin deficiency revealed 15 members with heterozygous MZ phenotypes. Levels of circulating alpha-1-antitrypsin and trypsin-inhibiting capacity were shown to be unreliable in identifying the heterozygous state, Pi typing being necessary for definitive diagnosis. The morphologic evolution of the hepatic changes in this condition have been studied, and the importance of the PAS stain in identification of the characteristic cytoplasmic bodies is stressed.- - - - - - - - - - ranking = 1.3911900065746keywords = member (Clic here for more details about this article) |
9/162. Use of radiation therapy in posttransplant lymphoproliferative disorder (PTLD) after liver transplantation.Posttransplant lymphoproliferative disorder (PTLD) is a common and life-threatening complication of immunosuppression used to prevent rejection of solid organ and bone marrow transplants. There is no standardized treatment algorithm, but numerous management strategies are available. We describe a patient who developed a solitary lymphoproliferative lesion in the porta hepatis 9 months after orthotopic liver transplant. Following reduction in immunosuppression with no response, she was treated with involved field radiotherapy utilizing CT-based treatment planning. A partial radiographic response was obtained, and she has not developed disease in the engrafted liver or systemically. Based on the present case report, involved field radiotherapy seems to be a reasonable treatment option for patients with localized PTLD. Int. J. Cancer (Radiat. Oncol. Invest.) 90:104-109, 2000.- - - - - - - - - - ranking = 7keywords = life (Clic here for more details about this article) |
10/162. Indian childhood-like cirrhosis in three Saudi Arabian siblings.Three Saudi siblings, two girls and one boy, presented at the ages of 7, 6 and 2.5 years, respectively, and were diagnosed as having features of Indian childhood cirrhosis (ICC). The two girls presented at a late stage of the disease and the boy was diagnosed during routine examination of the family. The initial presenting complaint was abdominal distention and pruritus. All three had a rapid and fatal course. There was no evidence of increased copper ingestion by the families, supporting the suggestion of a hereditary metabolic role in the aetiology of ICC. As far as we are aware, this is the first report of ICC in Saudi Arabian children.- - - - - - - - - - ranking = 62.439947850447keywords = family (Clic here for more details about this article) |
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