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Liver Involvement (liver + involvement)
Selected AbstractsLiver transplantation for cardiac failure in patients with hereditary hemorrhagic telangiectasiaLIVER TRANSPLANTATION, Issue 7 2005Thierry Thevenot Liver involvement in hereditary hemorrhagic telangiectasia may lead to high-output cardiac failure. Few data have been reported on orthotopic liver transplantation (OLT) for these patients. In this paper, we describe two patients treated by OLT as a salvage procedure for cardiac failure, and we review literature on this subject. Our two patients resumed normal cardiac function after OLT. This procedure appears to be a promising therapy with good long-term results despite dissection difficulties encountered due to the collateral arterial network reorganization. (Liver Transpl 2005;11:834,838.) [source] Hepatitic pattern of graft versus host disease in childrenPEDIATRIC BLOOD & CANCER, Issue 5 2007Héctor Melín-Aldana MD Abstract Background Liver involvement by graft-versus-host disease (GVHD) is characterized histologically by bile duct damage, which may be severe. A different pattern, "hepatitic GVHD," has been described in adult patients. This pattern also shows marked lobular hepatitis and hepatocellular damage. We report the development of hepatitic GVHD in six pediatric patients. Procedure Clinical information and histologic features of liver biopsy samples were retrospectively reviewed. Results Patients' ages ranged from 3 to 11 years. Underlying diagnosis, pre-transplant conditioning and GVHD prophylaxis varied. Peripheral blood stem cells were the source of the allograft in four patients, matched sibling in one, and matched-unrelated donor in one. Hepatic GVHD was detected between 149 and 310 days post-transplant. Prior acute GVHD had developed in two patients, and involved the skin and/or gastrointestinal tract. No patients had significant ductopenia. Only one patient had significant lymphocytic infiltration of bile ducts (ductitis). Bile duct epithelial damage and significant portal/periportal inflammation were present in all patients. Lobular necro-inflammation was present in five patients. Five patients improved with immunosuppression and one died with progressive GVHD. Conclusions This series focuses on hepatitic GVHD in pediatric patients. Clinical and histologic patterns are similar to what has been described in adults. Specific etiology and pathogenesis of this entity remain unclear. Pediatr Blood Cancer 2007;49:727,730. © 2006 Wiley-Liss, Inc. [source] Persistent parvovirus B19 infection detected by specific CD4+ T-cell responses in a patient with hepatitis and polyarthritisJOURNAL OF INTERNAL MEDICINE, Issue 3 2009G. Pongratz Abstract. We, here, report the case of a parvovirus B19 infection in an immunocompetent male patient presenting with acute hepatitis and polyarthritis. To follow the course of infection, we used a previously established enzyme-linked immunosorbent spot assay (ELISPOT) technique to detect CD4+ T cells specific for viral proteins. Even though symptoms of arthritis and hepatitis resolved in the immunocompetent individual within a few weeks, viral DNA in serum and CD4+ T cells specific for the viral protein VP1 unique region were still detectable more than 6 month after the onset of symptoms, thus pointing to a persistent state of infection. On the basis of this observation, we hypothesize that the intensity of liver involvement correlates with the likelihood of developing persistent parvovirus B19 infection. The described ELISPOT technique to detect virus-specific CD4+ T cells provides an excellent tool to analyse the state of parvovirus B19 infection for future studies to test this hypothesis. [source] Hepatic artery chemoembolization for 110 gastrointestinal stromal tumorsCANCER, Issue 12 2006Response, prognostic factors, survival Abstract BACKGROUND. The efficacy of hepatic artery chemoembolization (HACE) was evaluated for gastrointestinal stromal tumors (GISTs) metastatic to the liver. METHODS. Records for patients with metastatic GIST who underwent HACE between January 1993 and March 2005 were reviewed and cross-sectional images were used to determine objective tumor response. Progression-free survival in the liver (PFS-liver) and overall survival (OS) were calculated with the Kaplan,Meier method. Patient, tumor, and treatment variables were analyzed to identify factors influencing survival. RESULTS. Of the 110 patients identified, the radiologic response to HACE could be evaluated in 85 patients, 12 of whom (14%) demonstrated partial responses, 63 of whom (74%) demonstrated stable disease, and 10 of whom (12%) demonstrated progressive disease. PFS-liver rates were 31.2%, 8.2%, and 5.4% at 1, 2, and 3 years, respectively; the median PFS time was 8.2 months. OS rates were 62% at 1 year, 32% at 2 years, and 20% at 3 years; the median OS time was 17.2 months. Patients who had >5 liver metastases and received only 1 HACE treatment were found to have a shorter PFS compared with patients with fewer metastases or those who received ,2 HACE sessions. Extensive liver involvement, the presence of extrahepatic metastases, and progression of liver disease after HACE were associated with poor OS. Use of imatinib prolonged OS time. CONCLUSIONS. HACE produced a durable tumor response or disease stabilization in the majority of patients with GISTs metastatic to liver. Extent of liver disease, presence of extrahepatic disease, number of embolization treatments, and use of imatinib were found to have prognostic influence on PFS, OS, or both. Cancer 2006. © 2006 American Cancer Society. [source] Hepatic arterial embolization and chemoembolization for the treatment of patients with metastatic neuroendocrine tumorsCANCER, Issue 8 2005Variables affecting response rates, survival Abstract BACKGROUND The objective of this study was to determine the prognostic variables that influence response and survival in patients with metastatic neuroendocrine tumors who are treated with hepatic arterial embolization (HAE) or chemoembolization (HACE). METHODS Patients with metastatic neuroendocrine tumors who underwent HAE or HACE were included in this retrospective study. Follow-up imaging studies were compared with baseline imaging to determine the radiologic response. Progression-free survival (PFS) and overall survival (OS) were calculated using the Kaplan,Meier method. Univariate and multivariate analyses were performed to assess the prognostic variables that affected response and survival. RESULTS The study included 69 patients with carcinoid tumors and 54 patients with pancreatic islet cell carcinomas. Patients who had carcinoid tumors had a higher response rate (66.7% vs. 35.2%; P = 0.0001) and had longer PFS (22.7 mos vs. 16.1 mos; P = 0.046) and OS (33.8 mos vs. 23.2 mos; P = 0.012) compared with patients who had islet cell carcinomas. For patients with carcinoid tumors, multivariate analysis identified male gender as the only independent risk factor for poor survival (P = 0.05). Octreotide was predictive marginally for PFS (P = 0.06). Patients who were treated with HAE had a higher response rate than patients who were treated with HACE (P = 0.004). For patients with islet cell carcinoma, an intact primary tumor, , 75% liver involvement, and extrahepatic metastases were associated with reduced OS in the univariate analysis; the presence of bone metastases was the only risk factor (P = 0.031) in the multivariate analysis. Patients who were treated with HACE had a prolonged OS (31.5 mos vs. 18.2 mos) and improved response (50% vs. 25%) compared with patients who were treated with HAE, although the differences did not reach statistical significance. CONCLUSIONS Patients with carcinoid tumors had better outcomes than patients with islet cell carcinomas. The addition of intraarterial chemotherapy to HAE did not improve the outcome of patients with carcinoid tumors, but it seemed to benefit patients with islet cell carcinomas. In patients who had carcinoid tumors, male gender predicted a poor outcome, and a trend toward prolonged PFS was observed in patients who received concomitant octreotide. An intact primary tumor, extensive liver disease, and bone metastases were associated with reduced survival in patients with islet cell carcinomas. Cancer 2005. © 2005 American Cancer Society. [source] Liver cirrhosis in an infant with Chanarin,Dorfman syndrome caused by a novel splice-site mutation in ABHD5ACTA PAEDIATRICA, Issue 10 2010M Cakir Abstract We described a Turkish girl with Chanarin,Dorfman syndrome who developed liver cirrhosis in the early infancy. She had all the clinical features of Chanarin,Dorfman syndrome such as ichthyosis, Jordan's anomaly, fatty liver disease and mild ectropion. The diagnosis was confirmed with a novel ABHD5 mutation. Liver steatosis or steatohepatitis with or without hepatomegaly is the predominant finding of Chanarin,Dorfman syndrome. Cirrhosis has been reported in patients with long-duration disease. Conclusion:, Local factors or dysfunction of local proteins such as mutations or polymorphisms in hepatic microsomal lipase and arylacetamide deacetylase may contribute the severity of liver involvement, and steatosis may progress to cirrhosis in the early infancy in Chanarin,Dorfman syndrome. [source] Treatment of severe acute graft-versus-host disease with anti-thymocyte globulinCLINICAL TRANSPLANTATION, Issue 3 2001Mats Remberger Severe acute graft-versus-host disease (GVHD) is one of the major complications after haematopoietic stem-cell transplantation (HSCT). Treatment of severe GVHD is difficult and the condition is often fatal. One proposed method of improving the therapy is to include anti-thymocyte globulin (ATG). Here, we will report our results in 29 patients using ATG as part of treatment for severe steroid-resistant acute GVHD. Four patients suffered from grade II, 13 from grade III and 12 from grade IV GVHD. Median time to grade II GVHD was 24 d (range 7,91 d) and to grade III was 29 d (range 8,55 d) after HSCT. Five different ATG preparations were used, rabbit ATG (R-ATG), BMA 031, OKT® -3, ATG-Fresenius and Thymoglobuline®. All patients had skin involvement, 26 also had gut involvement and 25 had liver involvement. The rate of response to treatment was best in skin involvement (72%), while liver and gut involvement showed lower response rates (38%). Eleven patients survived more than 90 d, 7 of them developed chronic GVHD, 1 developed mild GVHD, 1 developed moderate GVHD and 5 developed severe GVHD. Survival at 100 d was 37% and at 1 yr it was 12%. Most patients died of GVHD, with virus or fungal infections as contributing causes of death. To conclude, treatment of severe acute GVHD is difficult and ATG, in our hands, adds nothing to conventional pharmacological treatment. [source] | ||||||||||||||||||||||