[Liver transplant outcomes in Brno]. / Výsledky transplantací jater v Brne

The first liver transplant in the former Czechoslovakia took place in Brno on 2 February 1983; the patient concerned has lived for more than 30 years with a normally functioning liver and is one of the longest surviving patients after a liver transplant in Europe. The Cardiovascular and Transplantation Surgery Centre (CTSC) in Brno has experienced an increased development in the area of liver transplants since the mid  1990s. At present, about 30 patients a year undergo a transplantation, with 451 liver transplants in total as of 31 December 2012. The primary indication concerns liver cirrhosis of various etiologies, with an increasing number of cases of cirrhosis resulting from hepatitis C. Urgent liver transplants (for acute liver failure or primary dysfunction of first liver graft) amount to 11% of cases. There were 18 retransplants performed as of 31 December 2012, with 50% five year survival. The primary graft dysfunction was present in 7 patients (i.e. 1.5%). The 1 year survival rate of all patients after a liver transplant performed in CTSC is 92%, 5 year survival rate is 80%, and 10 year survival rate is 71%. Currently the parameters such as recipients age, donors age, and transplant waiting time has been statistically increasing; small recipients with a body weight below 70 kg especially have to wait for a significantly longer period of time (waiting median of 178 days). In CTSC Brno 14 combined simultaneous transplants (13 transplants of liver + kidney and one combined simultaneous transplants of liver + heart + kidney in 2005) have been performed as of 31 December 2012.

[Interventional radiology options to address certain complications of the orthotopic liver transplant]. / Moznosti intervencní radiologie pri resení ne-kte-rých komplikací ortotopické transplantace jater.

At the clinic of imaging method St. Annas University Hospital solved complications arising after ortotopic liver transplantation. For more than 15 years of cooperation with CKTCH Brno intervention was performed on both the arterial and venous system, but most on the biliary tract. The order was a unit patients, which correlates with other comparable work. In the years 1998- 2013 we conducted one intervention on arterial bed, 3× intervention in hepatic venous system and we solved biliary complications in 7 transplant. .

[Liver transplants in tumours and alcoholic cirrhoses]. / Transplantace jater u tumoru a alkoholických cirhóz.

The treatment of hepatocellular carcinoma requires a multidisciplinary approach; liver transplant is suitable only in the minority of strictly selected patients. The CSTC Brno applies the so  called Milan criteria. As of 31 December 2012 the CSTC Brno performed liver transplants in 16 patients with hepatocellular carcinoma in cirrhosis, with a five year survival rate of 40% and a 10 year survival after transplant of 20%. It is a paradox that the longest living (30 years) patient of CSTC Brno underwent the transplant for a large fibrolamellar hepatocellular carcinoma, which emphasises the prognostic significance of the tumour histology -  criterion taken into account for practical reasons only in some indication schemes. Liver transplant for cholangiocarcinoma is no longer being carried out in CSTC. Five patients underwent this operation in the past and the longest living one survived for four years after the transplant. Benign liver tumours (adenomatosis, cystadenoma, hemangioma with compression symptoms) are rather rare indications and transplant results are positive. Four patients were transplanted for liver carcinoid, with one patient experiencing recurrence. The most frequent compact indication for liver transplant in CSTC Brno concerns alcoholic cirrhosis (24%, 108 patients); the survival rate of these patients after transplant is very good (81% 5 year survival and 68% 10 year survival). Likewise, efforts are made to select patients with a low risk of alcohol abuse after the transplant, which is, however, not very successful. The recurrence of some kind of alcohol abuse occurred in 26% transplanted patients, while seven died of alcoholic cirrhosis of the graft.

Wilson's disease: monocentric experiences over a period of 10 years [corrected].

BACKGROUND: Wilson's disease (WD) is an autosomal recessive disorder of copper metabolism. The objective of this study is to present diagnostic pitfalls and long time follow-up data in Wilson disease. PATIENTS/METHODS: We studied 21 WD patients and 14 heterozygote carriers aged 2-43 years, retrospectively. 18 WD patients presented liver disease, three had mixed neurological and hepatic involvement and 9 patients underwent orthotopic liver transplantation (OLT). RESULTS: The median age at diagnosis of WD children without OLT was 10.16+/-3.8 (range, 5-16). All of females and younger age categories of patients prevailed in acute liver failure group. Serum ceruloplasmine levels were below 0.2 g/l in about (1/3) of WD carriers (X =0.27+/-0.09 g/l) and nearly (2/3) of children with WD (X = 0.21+/-0.13 g/l). A statistically significant difference (p<0.05) in the 24-h excretion of copper in urine was noticed between healthy controls, children with WD and WD heterozygote carriers. As diagnostic important proved the copper content of more than 250 microg/g hepatic dry weight. The Kayser-Fleischer?s ring was not observed in children. Ceruloplasmine, haemoglobin, ALT, ALP and plasma albumin were significantly different between fulminant and non-fulminant WD and could be used as indirect markers in evaluation of urgent OLT. CONCLUSION: Detection of WD in children remains very difficult. The most important investigation is liver biopsy with the assessment of liver copper. Genetic analysis may help in doubtful cases.

Haemolytic anaemia and acute liver failure--the initial manifestations of Wilson's disease.

We describe a case of a 16-year-old girl with Wilson disease, which was initially presented as Coombs-negative haemolytic anaemia and acute liver failure. The diagnosis was based on the findings of low ceruloplasmin serum level and high copper levels both in serum and 24-hour urinary excretion. The patient underwent orthotopic liver transplantation. A DNA-based diagnostic tool confirmed Wilson's disease: the patient was p.H1069Q homozygote. Based on further molecular-genetic examinations in the family, Wilson disease was diagnosed seven days later in one of the patient's asymptomatic brothers. The proband's cousin was confirmed as a carrier of the p.H1069Q mutation (Fig. 1, Ref. 24).

[Liver transplantation for hepatitis B]. / Transplantace jater pro hepatitidu B.

Up to now the outcomes of liver transplantation in patients with chronic viral hepatitis B have not been very good because the recurrence of viral hepatitis in the graft has been high and resulted in a high early graft failure of liver transplant recipients. However, the administration of a combined therapy with lamivudine and hyperimmune anti-HBs globulin has led to a marked improvement in transplantation results and an increase in the number of liver transplantations for this indication. Four men (aged 47 to 55 years) underwent liver transplantation for cirrhosis, caused by chronic viral hepatitis B, at our centre. All were HBsAg carriers. They were our first patients who received therapy with the combined immunoprophylactic regimen of lamivudine and hyperimmune anti-HBs globulin. HBV DNA negativity was achieved in all patients prior to transplantation; three of them were pretreated with lamivudine. At 4 to 17 months of follow-up, sustained suppression of HBV replication (HBV DNA negativity) was maintained in all four patients. No complications associated with this treatment were observed and no emergence of resistant mutants was detected. The combined therapy for chronic viral hepatitis B administered to liver transplant recipients at our centre showed very good outcomes. However, the development of resistant mutants during this therapy poses a problem, which may hopefully be overcome with the use of new antivirotics, such as adefovir or tenofovir.