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Van Keimpema L.,Radboud University Nijmegen | Nevens F.,University Hospital euven | Adam R.,Center Hepato Biliaire | Porte R.J.,University of Groningen | And 5 more authors.
Transplant International | Year: 2011

Summary Patients with end-stage isolated polycystic liver disease (PCLD) suffer from incapacitating symptoms because of very large liver volumes. Liver transplantation (LT) is the only curative option. This study assesses the feasibility of LT in PCLD. We used the European Liver Transplant Registry (ELTR) database to extract demographics and outcomes of 58 PCLD patients. We used Kaplan-Meier survival analysis for survival rates. Severe abdominal pain (75%) was the most prominent symptom, while portal hypertension (35%) was the most common complication in PCLD. The explantation of the polycystic liver was extremely difficult in 38% of patients, because of presence of adhesions from prior therapy (17%). Karnofsky score following LT was 90%. The 1- and 5-year graft survival rate was 94.3% and 87.5%, while patient survival rate was 94.8% and 92.3%, respectively. Survival rates after LT for PCLD are good. © 2011 The Authors.

Di Dalmazi G.,Ludwig Maximilians University of Munich | Kisker C.,University of Wurzburg | Calebiro D.,University of Wurzburg | Arnaldi G.,University Hospital | And 15 more authors.
Journal of Clinical Endocrinology and Metabolism | Year: 2014

Context: Somatic mutations in PRKACA gene, encoding the catalytic subunit of protein kinase A (PKA), have been recently found in a high proportion of sporadic adenomas associated with Cushing's syndrome. The aim was to analyze the PRKACA mutation in a large cohort of patients with adrenocortical masses.Methods: Samples from nine European centerswere included (Germany, n = 4; Italy, n = 4; France, n = 1). Samples were drawn from 149 patients with nonsecreting adenomas (n = 32 + 2 peritumoral), subclinical hypercortisolism (n = 36), Cushing's syndrome (n = 64 + 2 peritumoral), androgen-producing tumors (n = 4), adrenocortical carcinomas (n = 5 + 2 peritumoral), and primary bilateral macronodular adrenal hyperplasias (n = 8). Blood samples were available from patients with nonsecreting adenomas (n = 15), subclinical hypercortisolism (n = 10), and Cushing's syndrome (n = 35). Clinical and hormonal data were collected. DNA amplification by PCR of exons 6 and 7 of the PRKACA gene and direct sequencing were performed.Results: PRKACA heterozygous mutations were found in 22/64 samples of Cushing's syndrome patients (34%). No mutations were found in peritumoral tissue and blood samples or in other tumors examined. The c.617A>C (p.Leu206Arg) occurred in 18/22 patients. Furthermore, two novel mutations were identified: c.600-601insGTG/p.Cys200-Gly201insVal in three patients and c.639C>G+c.638-640insATTATCCTGAGG/p.Ser213Arg + p.Leu212-Lys214insIle-Ile-Leu-Arg) in one. All the mutations involved a region implicated in interaction between PKA regulatory and catalytic subunits. Patients with somatic PRKACA mutations showed higher levels of cortisol after dexamethasone test and a smaller adenoma size, compared with nonmutated subjects.Conclusions: These data confirm and extend previous observations that somatic PRKACA mutations are specific for adrenocortical adenomas causing Cushing's syndrome. Copyright © 2014 by the Endocrine Society.

Spatz H.,Visceral and Transplant Surgery | Anthuber M.,Visceral and Transplant Surgery
European Surgery - Acta Chirurgica Austriaca | Year: 2010

Background: Colon cancer is a leading cause of death in western countries and accounts for 639,000 cancer-related deaths worldwide. Surgical tumour clearance is the key treatment option and represents a classic field of action for the general and visceral surgeon. Methods: PubMed, EMBASE and DIMDI were searched for articles referring to state of the art surgical management of colon cancer complemented by our own clinical experience to create an overview of up-to-date treatment options and theoretical background knowledge. Results: The extent of bowel and mesenteric resection is dictated by the vascular supply and corresponding lymphatic drainage, careful attention to anatomical-embryological tissue planes and provision of adequate blood supply at the resection margins. Accordingly, optimal surgical clearance results in low local recurrence rates and translates into improved survival. In this context, the open and laparoscopic approaches yield similar results in terms of long-term outcome and oncological radicality. Radical resection of metastases results in long-term survival in selected patients. Fast Track rehabilitation facilitates attenuation of perioperative stress response and enhances recovery. Conclusion: Resectability and surgical technique are pivotal for long-term survival in all stages of colon cancer. © Springer-Verlag 2010.

Ollinger R.,Innsbruck Medical University | Mihaljevic A.L.,TU Munich | Schuhmacher C.,TU Munich | Bektas H.,Visceral and Transplant Surgery | And 7 more authors.
HPB | Year: 2013

Background: Bleeding during hepatic surgery is associated with prolonged hospitalization and increased morbidity and mortality. The Veriset™ haemostatic patch is a topical haemostat comprised of an absorbable backing made of oxidized cellulose and self-adhesive hydrogel components. It is designed to achieve haemostasis quickly and adhere to tissues without fixation. Methods: A prospective, randomized, multicentre, single-blinded study (n = 50) was performed to compare the use of a Veriset™ haemostatic patch with a fibrin sealant patch (TachoSil®) (control) in the management of diffuse bleeding after hepatic surgery. Patients were randomized following the confirmation of diffuse bleeding requiring the use of a topical haemostat. Time to haemostasis was assessed at preset intervals until haemostasis was achieved. Results: Both groups were similar in comorbidities and procedural techniques. The median time to haemostasis in the group using the Veriset™ haemostatic patch was 1.0 min compared with 3.0 min in the control group (P 0.001; 3-min minimum application time for the control patch). This result was independent of bleeding severity and surface area. Both products had similar safety profiles and no statistical differences were observed in the occurrence of adverse or device-related events. Conclusions: Regardless of bleeding severity or surface area, the Veriset™ haemostatic patch achieved haemostasis in this setting significantly faster than the control device in patients undergoing hepatic resection. It was safe and easy to handle in open hepatic surgery. © 2012 International Hepato-Pancreato-Biliary Association.

Eurich D.,Visceral and Transplant Surgery | Bahra M.,Visceral and Transplant Surgery | Berg T.,University of Leipzig | Boas-Knoop S.,Visceral and Transplant Surgery | And 4 more authors.
Experimental and Clinical Transplantation | Year: 2011

Objectives: Hepatitis C-virus-persistence after orthotopic liver transplant leads to reduced patient and graft survival compared to other indications. Current interferon-based antiviral therapy of hepatitis C-virus-infection posttransplant provides a sustained response rate of 30% to 40%. This study, performed in an hepatitis C-virus-reinfected liver transplant population, examines the antiviral effect of intravenously administered silibinin, recently reported to exhibit strong antiviral properties in the natural setting of hepatitis C-virus-related liver disease. Patients and Methods: Four patients after orthotopic liver transplant with hepatitis C-virus- recurrence, previously having not responded to peg-interferon-ribavirin therapy, were treated with intravenous silibinin and additionally, after the 10th day, with standard interferon-based therapy. Aminotransferases and hepatitis C-virus-RNA were measured during treatment. Results: All patients demonstrated normalization of liver enzymes and significant decline of hepatitis Cvirus- RNA measured at day 10 (mean 2.8 logarithmic levels: 1.7, 2.3, 2.9, and 4.3) during silibinin monotherapy. One patient cleared hepatitis C-virus-RNA under silibinin monotherapy and another patient eliminated hepatitis C virus under subsequent interferon-based therapy. No adverse effects were observed during silibinin application. Conclusions: Intravenous silibinin is an effective therapeutic approach for treating hepatitis C-virus- reinfection after liver transplant and should be evaluated further. © Başkent University 2011.

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