Fibrosing Cholestatic Hepatitis C After Liver Transplantation: Therapeutic Options Before and After Introduction of Direct-Acting Antivirals: Our Experience and Literature Review.

BACKGROUND: Cirrhosis caused by hepatitis C is the most common indication for liver transplantation. The most aggressive form of hepatitis C virus (HCV) relapse after liver transplantation is fibrosing cholestatic hepatitis C, which can be observed in 2% to 15% of recipients. METHODS: Double therapy with peg-interferon and ribavirin was characterized by low antiviral response, rapid fibrosis, and frequent graft failure within 1 year after surgery. RESULTS: Introduction of direct-acting antivirals for HCV treatment allows for more efficient therapy with less adverse reactions, including patients with fibrosing cholestatic hepatitis C. CONCLUSIONS: We present 4 (2.5%) cases of cholestatic viral hepatitis C recurrence in patients undergoing transplantation between 2006 and 2015 at the Transplantation Institute of Warsaw; during this period, 158 liver transplants were performed in patients with cirrhosis caused by HCV infection.

Delayed, Uncommon Recurrence of Hepatocellular Carcinoma After Liver Transplantation: A Case Report.

We report a case of a 27-year-old man diagnosed with the infection of HBV delta in the 8th month of life. The treatment complied with evidence-based medical guidelines, comprising neoadiuvant chemotherapy and surgery. Liver transplantation from a deceased donor followed by chemotherapy was performed when the patient was 16 years 9 months of age because of recurrent HCC tumor. The patient qualified for immunosuppressive treatment (rapamycin, tacrolimus), lamivudine, anti-HBs globulin intravascular infusion, and anti-HBV vaccination as a prophylaxis against reinfection with HBV. In conclusion, this case demonstrates the importance of a postoperative follow-up of patients with HCC, even years after liver transplantation.

The effects of in vitro hemodilution and fibrinogen concentrate substitution on thromboelastometry analysis in patients qualified for liver transplantation - preliminary results.

BACKGROUND: Dilutional coagulopathy might cause life-threatening hemorrhages in liver transplantation. Liver insufficiency is usually accompanied by alteration in fibrinogen (Fib) synthesis, which is one of the main clotting factors providing appropriate hemostasis. Intraoperative hemodilution results in further Fib concentration reduction enhancing coagulopathy and blood loss. Exogenous Fib substitution might prevent this. METHODS: A prospective study with a control group was designed. The study group consists of patients with cirrhosis who qualified for liver transplantation. Inclusion and exclusion criteria were strictly established. The blood collected from participants was diluted up to 30% and 60% with crystalloid (saline) or colloid (hydroxyethyl starch) in 2 parallel series. The first series consisted of diluted blood, the second of diluted blood with Fib concentrate. Thromboelastometry tests were performed on every blood sample. After collecting data from the first 12 participants, we performed a preliminary analysis. RESULTS: The maximum clot formation (MCF) in the EXTEM test decreased with progressive blood dilution in both study arms. The MCF values were lower than 35 mm in every diluted blood sample of the study group. The recovery of decreased MCF after Fib concentrate substitution was observed in both groups. The improvement in clot formation was also expressed as amplitude of clot firmness in the 10th minute (A10) in the FIBTEM test. CONCLUSIONS: Clot formation is disturbed more profoundly by hemodilution in cirrhotic patients. Fib concentrate substitution might be effective in the management of dilutional coagulopathy.

Bacterial and fungal infections in the early post-transplantation period after liver transplantation: etiologic agents and their susceptibility.

OBJECTIVE: It has been reported in many studies that one of the main factors influencing morbidity and mortality in patients receiving transplants is infection after transplantation. PATIENTS AND METHODS: The study included 190 adult patients undergoing orthotopic liver transplantation (OLT) between September 2001 and December 2007. All the patients were followed prospectively for infections from the OLT date and during the first 4 weeks after surgery. Immunosuppression consisted of steroids and tacrolimus. Antimicrobial prophylaxis included piperacillin/tazobactam, fluconazole, and selective bowel decontamination (SBD) was performed. Samples of clinical materials were investigated for microbiological cultures. The micro-organisms were cultured and identified in accordance with standard bacteriological procedures. Susceptibility testing was performed using Clinical and Laboratory Standards Institute procedures. RESULTS: From 190 OLT recipients, 2213 clinical samples were obtained for microbiological examination. Positive cultures were found in 27.2% (n = 603) of all samples tested; 1252 strains were collected. Gram-positive bacteria were found in 64.1% (n = 802), Gram-negative bacteria were found in 31.6% (n = 396), and fungal strains were isolated in 4.3% (n = 54). Surgical site specimens (n = 1031) were obtained from 190 recipients during the first month after transplantation. Positive cultures accounted for 29.2% (n = 301) of all samples tested. Among the isolated microbial strains (n = 677), most common were Gram-positive bacteria (73.7%; n = 499). Gram-negative bacteria comprised 25.1% (n = 170). There were fungal strains in 1.2% (n = 8). There were 539 urine specimens. Positive cultures accounted for 16.7% (n = 90) of those. Among the isolated microbial strains (n = 210), most common were Gram-negative bacteria (62.4%; n = 131). Gram-positive bacteria comprised 28.6% (n = 60) and fungi 9% (n = 19). There were 549 blood specimens. Positive cultures were found in 30.6% (n = 168) of all samples tested. Among the isolated microbial strains (n = 263), most common were Gram-positive bacteria in 72.3% (n = 190); Gram-negative bacteria were found in 26.2% (n = 69), and fungal strains were isolated in 1.5% (n = 4). There were 69 respiratory tract specimens. Positive cultures were found in 46.4% (n = 32) of all samples tested. Among the isolated microbial strains (n = 84), most common were Gram-positive bacteria (51.2%; n = 43); Gram-negative bacteria comprised 27.4% (n = 23) and fungi 21.4% (n = 18). CONCLUSIONS: (1) Surgical site samples were predominated samples after LTx. (2) Our study showed Gram-positive bacteria were 64.1% (n = 802), Gram-negative bacteria, 31.6% (n = 396) and fungal strains isolated in 4.3% (n = 54). (3) The increased proportion of isolates of multi-drug-resistant bacterial strains (methicillin resistant coagulase negative Staphylococcus, vancomycin-resistant Enterococcus, high-level aminoglycoside resistance, and extended- spectrum ß-lactamase). (4) These data indicate strict cooperation infection control procedures in these patients.

Urinary tract infections in the early posttransplant period after liver transplantation: etiologic agents and their susceptibility.

BACKGROUND: We performed an analysis of etiologic agents for urinary tract infections in the early posttransplant period after orthotopic liver transplantation (OLT) in adult recipients. PATIENTS AND METHODS: The study covered the first 4 weeks after OLT of 190 patients from September 2001 to the end of 2007. Immunosuppression consisted of steroids and tacrolimus. Antimicrobial prophylaxis was piperacillin/tazobactam, fluconazole, and SBD. Urine samples were cultured to identify microorganisms in accord with standard microbiological procedures and to test susceptibility using Clinical Laboratory and Standards Institute guidelines. RESULTS: Urine specimens (n=539) examined from 185 recipients (97.4%) showed 210 microbial strains. The most common were Gram-negative (n=131; 62.4%) with predominance of Escherichia coli (28.2%), Enterobacter cloacae (19.1%), and Acinetobacter baumannii (11.4%). Extended-spectrum ß- lactamases (ESBL(+)) strains were isolated in 38.5% of cases. Gram-positive bacteria comprised 28.6% (n=60): The most common strains were enterococci (85% including HLAR 80.4% and VRE 17.6%] and staphylococci 11.8% [MRSA/MRCNS; 100%]. There were 19 (9%) fungal strains. CONCLUSIONS: In general, the identification in urine samples of multi-drug-resistant bacterial and fungal strains in patients after OLT such as ESBL(+) 38.5%; HLAR 80.4%; VRE 17.6%; and MRSA/MRCNS 100% requires better infection control.

Detrimental effect of aprotinin ban on amount of blood loss during liver transplantation: single-center experience.

BACKGROUND: Aprotinin, a plasmin inhibitor, had been used for reduction of intraoperative bleeding caused by hyperfibrinolysis during extensive surgery. Prophylaxis with aprotinin to limit blood loss during orthotopic liver transplantation (OLT) had been widely applied until the drug was weaned off the therapeutic list for severe complications. We compared the need for blood and blood products transfusion in patients undergoing OLT with and without the use of aprotinin. MATERIALS AND METHODS: A retrospective analysis was performed on 150 patients, who underwent OLT between March 2004 and August 2008 and were divided into 2 groups: the APRO group (n = 111) after induction of anesthesia was given a bolus of 500 kIU of aprotinin in a 30-minutes infusion followed by 140 kIU/h till the end of the OLT in which aprotinin was not administered, and the NON-APRO group (n = 39). RESULTS: Patients from the NON-APRO group needed significantly more units of packed red blood cells (PRBC) than the APRO group (5.53 ± 4.89 vs 3.99 ± 3.58 units; P = .037). Avoidance of aprotinin administration (ß = 1.408), Child-Pugh score (ß = 0.519), and duration of anhepatic phase (ß = 0.03) affected the volume of transfused blood according to multiple regression analysis (P < .05). CONCLUSIONS: Our study confirmed the important prophylactic role aprotinin used to have during OLT in limiting the need for blood transfusions. Further research and progress in methods of blood loss minimization and monitoring of hemostasis are needed to warrant safe liver transplantation.

CTX-M and TEM as predominant types of extended spectrum beta-lactamases among Serratia marcescens isolated from solid organ recipients.

BACKGROUND: Serratia marcescens is an important pathogen in hospital infections since organisms resistant to multiple antimicrobials pose a special threat particularly among transplant patients. The aim of this work was to assess the number of strains producing beta-lactamases with extended spectrum (ESBL) among S. marcescens isolated from our patients. MATERIALS AND METHODS: We investigated S. marcescens isolated from 2005 to 2008 for ESBL. The phenotype methods were applied and additionally we chose strains for polymerase chain reactions using primers for the most popular types of ESBL. RESULTS: Over the investigated time, 257 patients were infected with S. marcescens with 188 (73%) displaying an ESBL-positive phenotype. A Molecular analysis showed that most of them produced both CTX-M and TEM beta-lactamases. In the last year, the percentage of ESBL-producing strains decreased, but also in the last year, we isolated S. marcescens resistant to carbapenems from three patients. CONCLUSIONS: The CTX-M type of ESBL predominated among ESBLs produced by strains of S. marcescens. The appearance of strains resistant to carbapenems is alarming.

Resistance to carbapenems among Pseudomonas aeruginosa isolated from patients of transplant wards.

BACKGROUND: The aim of the study was to estimate the carbapenems resistance and occurrence of metallo-beta-lactamase (MBL) production among Pseudomonas aeruginosa isolated from patients during the last 2 years. MATERIALS AND METHODS: We investigated all P aeruginosa strains derived from Transplantation Institute patients, hospitalized from January 2007 to December 2008. E-tests as well as the three-discs method with imipenem, ceftazidime were used for MBL detection. For the chosen strains, a PCR method was applied for detection of genes determining VIM, IMP, and SMP. RESULTS: Among 311 isolated strains from 228 patients only one strain was used for each patient. We showed increased resistance to carbapenems among P aeruginosa in 2008 compared with 2007: from 14% to 22%. About 60% of resistant strains displayed the MBL phenotype. Upon PCR analysis, the VIM-type metallo-beta-lactamase was detected in 70% of them. CONCLUSIONS: Despite similar numbers of P aeruginosa-infected patients in 2007 and 2008, the percentage of MBL-producing strains increased from 7% to 15%. Most MBLs belonged to the VIM type.

Surgical site infections in liver recipients in the early posttransplantation period: etiological agents and susceptibility profiles.

OBJECTIVE: This study evaluated the frequency of microbial isolates and their susceptibility profiles from cultures at the surgical site of 83 liver recipients in the early posttransplantation period. PATIENTS AND METHODS: We prospectively collected microbiologic culture data on 83 adult patients undergoing orthotopic liver transplantation (OLT) using standard procedures and commercially available tests. Susceptibility of the strains to antibacterial agents was performed by the Clinical and Laboratory Standards Institute (CLSI) guidelines. RESULTS: All patients were followed prospectively for the first 4 weeks after surgery. Among 284 microbial isolates from clinical surgical site samples in 80 liver recipients, cultures were positive in 110 samples. The most commonly isolated species were: Gram-positive cocci (n = 222 isolates, 78%) with dominance of methicillin-resistant coagulase-negative staphylococci (MRCNS; 42%) and high-level aminoglycoside-resistant enterococci (HLAR strains; 24.3%). Gram-negative bacteria were identified in 21.5% of positive cultures, including 30 strains (24%) from the Enterobacteriaceae family, with 13.3% of extended spectrum beta-lactamase producers [ESBL(+)]. Significant differences (P = .0012) were observed during the analysis of changes in the occurrence of Gram-positive bacteria isolated from the surgical site in the first week versus the second to the end of the fourth week. CONCLUSION: Gram-positive bacteria predominated as 78% of isolates.

Bacteria isolated from bile samples of liver recipients in the early period after transplantation: epidemiology and susceptibility of the bacterial strains.

OBJECTIVE: We estimated the frequency and susceptibility to antibacterial agents of bacterial isolates from bile samples obtained from 83 liver recipients in the early period after transplantation. PATIENTS AND METHODS: We prospectively collected data on 83 adult patients undergoing orthotopic liver transplantation (OLT), including bile samples taken during the first 30 days after OLT from adult liver recipients suspected to have bile infections. The isolation/identification of cultured bacteria was performed according to standard microbiological procedures and commercially available tests. Susceptibility of the strains to antibacterial agents was determined according to the Clinical and Laboratory Standards Institute (CLSI) guidelines. RESULTS: Among 210 bile samples obtained from 79 liver recipients, bacterial cultures were positive in 110 samples from 59 (75%) recipients yielding 156 bacterial strains. The most commonly isolated species were as follows: gram-positive cocci (109 isolates) with dominance of coagulase-negative staphylococci (52%) and enterococci (36%); and gram-negative bacteria, 21 strains from the Enterobacteriaceae family and 14 of non-fermenting rods. We identified some multidrug-resistant (MDR) bacterial strains. In the first week after OLT, we investigated samples from 59 patients, yielding 36 bacterial strains. From the second to the end of the fourth week after OLT, 120 bacterial strains were isolated from 65 recipients. CONCLUSION: Gram-positive bacteria comprised 68.5%. The dominance of MDR gram-positive bacteria may be related to selection by perioperative antibiotic prophylaxis.

Detection of Clostridium difficile in stool samples from patients in the early period after liver transplantation.

OBJECTIVE: We examined the frequency of detection of Clostridium difficile (CD) toxins compared with the recovery of C. difficile in stool specimen cultures among orthotopic liver transplant (OLT) patients with nosocomial diarrhea in the early period. MATERIALS AND METHODS: The study included stool samples obtained during the first 30 days after OLT in adults who were suspected of CD-associated diseases. The identification of cultured CD strains was performed by standard microbiological methods. The presence of CD toxins was assayed using a commercial immunoassay. RESULTS: All patients were followed prospectively for CD infections from the date of OLT for the first 4 weeks after surgery. Among 54 samples, 16.7% were culture-positive for CD. CD toxins were tested on 54 samples, yielding 63% toxin-positive samples and 30% toxin- and culture-negative results. In the first week after OLT, samples from 19 patients were subjected to CD investigation. Among 19 samples positive for toxin, 52.6% of all samples were culture-negative. We analyzed 35 samples from the second to the fourth week after OLT in 31 recipients. Among 35 samples, 68.6% and 25.7% were positive for CD toxin and for culture, while 20% of samples were negative for toxin and culture. CONCLUSION: In our study, 63% of samples were toxin-positive with 16.7% yielding growth of CD and 30% being negative for toxins and cultures.

Etiological agents of bacteremia in the early period after liver transplantation.

Bacteremia is one of the major infections in orthotopic liver transplantation (OLT). The study of 83 adults who underwent OLT from 2001 to 2004, included patients followed prospectively from the day of transplantation to 4 weeks after the procedure by bacteriological cultures. The microorganisms were investigated according to standard National Committee for Clinical Laboratory Standards (NCCLS) procedures. Blood samples were examined in 59 recipients (71.1%) before and in 76 patients (91.6%) during the month after transplantation. Among 249 investigated samples, 96 were positive, as cultured from 19 recipients before OLT and 48 patients afterward. The most common were Gram-positive cocci (n = 71) and coagulase-negative staphylococci (n = 52), including methicillin-resistant coagulase-negative staphylococci (MRCNS). Enterococcus spp. occurred in 9 isolates (high-level aminoglycoside-resistant enterococci [HLAR] strains were cultured). We cultured the Enterobacteriaceae family (n = 16 isolates) and (n = 15 isolates), Gram-negative nonfermenting rods some of which were extended spectrum beta-lactamase producing [ESBL(+)] strains. The predominance of Gram-positive cocci was caused by CNS, and the use of prophylaxis to reduce Gram-negative bacteria. The increased rate of isolation of bacteria with multidrug resistance (MDR) to antimicrobial agents may be due to their frequent use for prophylaxis of bacterial infections in OLT. These MDR bacterial strains caused severe BSI after OLT.

Intraoperative blood flow measurements in organ allografts can predict postoperative function.

A reliable method to recognize the extent of ischemia/reperfusion injury in transplantation is needed in order to tailor the immunosuppressive scheme to the needs of a damaged organ. This study sought to assess the correlation between the total and the parenchymal blood flow into a transplanted kidney (n = 71) or liver (n = 15) shortly after revascularization with the early function of the organ after transplantation. The total blood flow in the renal artery in kidney recipients or in the hepatic artery and portal vein in liver recipients was measured by an electromagnetic flowmeter. The parenchymal blood flow (in several parts of the transplanted organ) was assessed using a laser-Doppler flowmeter. Two measurements were always taken after revascularization (5 to 60 minutes apart). Vascular resistance (VR) as calculated by the difference between the mean arterial pressure (MAP) and the central venous pressure (CVP) was correlated with immediate kidney or liver function parameters. Neither total renal blood flow (RBF) nor VR was different between the immediate function (IF) and delayed graft function (DGF) groups of kidney transplant patients. However, the cortical (parenchymal) blood flow was significantly greater in the IF than the DGF group at 5 minutes: 29.98 +/- 6.13 mL/min/100 g vs 23.56 +/- 6.46 mL/min/100 g (P < .001). The difference was even more significant at 35 minutes: 33.94 +/- 7.47 mL/min/100 g vs 15.47 +/- 3.34 mL/min/100 g (P < .0001). Among liver transplant patients, the results suggested a correlation between hepatic arterial blood flow and early graft viability and function. The most reliable predictor of early graft function was the portal blood flow, which correlated with the volume of secreted bile as well as the bilirubin, and transaminase levels and coagulation profile. Further studies must confirm the value of measurements of total and parenchymal blood flow in organ transplant recipients.

Questionnaire regarding the knowledge of the rules of organ procurement from cadaveric donors for transplantation directed to the directors of medical institutions in Poland.

We assessed the level of knowledge of organ procurement regulations among the directors of medical institutions in Poland. We also sought to promote the objection form, and the activity of the Central Register of Objections. A questionnaire consisting of 10 questions was sent to 381 random medical health care institution directors countrywide. In 89% of surveyed institutions, the written text of the organ procurement regulations was available and 94% of directors knew the forms of objection, but in 26% of institutions the form was not available and in 14% it was never obtainable. In the medical institutions directors' opinions, the estimated number of objections is 13% of the population in Poland. Organ transplantation is a form of treatment most medical institutions are familiar with, but the matter of donation is not as well known as transplantation, as observed in 48% of questioned institutions.

Intraoperative blood flow measurements and liver allograft function: preliminary results.

INTRODUCTION: Our previous studies showed a correlation of intraoperative renal allograft blood flow and immediate functions. A similar relation is not well established for liver transplantation. The aim of this study was to assess the relation between hepatic blood flow on revascularization and immediate liver graft function (IF). METHODS: Studies evaluating arterial and portal flow in newly transplanted livers were started in May 2004. Total hepatic artery and portal vein blood flow were assessed in 15 liver transplant recipients. Parenchymal flow was also recorded. Measurements were taken at 30 and 120 minutes after simultaneous arterial/portal reperfusion. Flow results were correlated with IF. RESULTS: Mean arterial blood flow (ABF) was 16.3 mL/min/100 g in both measurements. Portal flow was reduced from 168 to 127 mL/min/100 g from the first to the second measurement. Mean parenchymal flow (PF) did not alter over time (29.1 and 30.4 mL/min/100 g, respectively). Among recorded flow results we observed a significant correlation between PF with IF measured as: bile volume (R = 0.36 to 0.62; P < .05), serum AST (R = -0.4 to -0.68; P < .05), and ALT level (R = -0.2 to -0.71; P < .05), bilirubin level as well as INR (R = -0.39 to -0.61; P < .05) assayed daily for 14 days. Similar observations were made between ABF and INR, hiatal parenchymal flow, and ALT as well as INR. CONCLUSIONS: These preliminary results suggest hepatic blood flow may be a reliable predictor of graft viability and function. Of the variables measured, portal blood flow seems to be the most valuable indicator of liver function.

Biliary complications following liver transplantation: single-center experience.

Biliary complications (BC) following orthotopic liver transplantation (OLT) remain one of the major causes of postoperative complications and treatment failures. The list of common BC consists of biliary stricture, fistula, ischemic type biliary lesions (ITBL), cholangitis, and bile leakage following T-drain removal. Between July 2000 and December 2004, 101 consecutive cadaveric OLTs were performed in our institution. All but three were first full-size grafts. Seventeen patients were transplanted from the urgent list, the remaining 84 (83.16%) from the elective list. All but three patients had a choledochocholedochostomy over a straight drain. Bile cultures were taken routinely. The bile drain was removed following cholangiography 6 weeks after OLT. All patients received antibiotic prophylaxis. Ursodeoxycholic acid was used in selected cases. During the first 6 weeks positive bile cultures in absence of clinical and biochemical symptoms of cholangitis were found in 61 (60.4%) cases. Symptomatic cholangitis requiring antibiotic treatment was observed in 19 (18.8%) patients during the first 6 weeks. Two patients required endoscopic sphincterotomy and temporary stenting due to anastomotic stricture (1) or papilla of Vater fibrosis (1). Bile leakage following drain removal was observed in 8 (7.9%) patients. Five of them were treated conservatively, the remaining 3 (2.9%) required surgery (lavage) and stenting. In one case extrahepatic bile duct necrosis was diagnosed requiring reconstruction of the biliary anastomosis. No case of ITBL, bile leak at the anastomostic site, or stricture requiring surgical repair was noted. Despite the high incidence of positive bile cultures most likely related to use of a drain, the overall number of BC was low.

Plasmacytic hyperplasia--the early form of posttransplant lymphoproliferative disorder--with atypical morphology and clinical course in patient after liver transplantation: a case report.

This case report describes an early lesion of posttransplant lymphoproliferative disorder (PLTD)--plasmacytic hyperplasia with atypical morphology. The 54-year-old patient was 4 months after liver transplantation due to alcoholic cirrhosis. The postoperative course had been uneventful without graft rejection episodes. Primary immunosuppressive therapy included tacrolimus and prednisone. On admission to the hospital the patient showed rapidly increasing jaundice, hepatomegaly, anemia, thrombocytopenia, and significant leukocytosis. A biopsy suggested generalized infection. Acute Epstein-Barr virus (EBV) infection was confirmed using serological methods. Despite treatment the patient died. On autopsy we found features of generalized infection. Histological examination of the enlarged lymph nodes showed plasmacytic hyperplasia despite lymph node atrophy. Plasmacytic hyperplasia, an early lesion of PTLD despite usually a good prognosis with multifactor therapy may display a rapid course that leads to death through intensified immunosuppression. In accordance with other reports we confirmed reactivation of EBV infection as the probable cause of plasmacytic hyperplasia. The lymph node morphology of plasmacytic hyperplasia may be atypical with atrophy of lymphoid components accompanying plasma cell proliferation.

Perioperative use of recombinant activated factor VII in liver transplantation.

Recombinant activated factor VII (rFVIIa, NovoSeven, Novo Nordisk A/S, Denmark) is a treatment used to prevent and arrest intra- and postoperative bleeds in patients with haemophilia A or B complicated by circulating anticoagulants (inhibitors of FVIII and FIX). Patients who qualify for liver transplantation may have varying degrees of coagulation impairment, which may adversely impact elective anaesthetic and surgical procedures and elevate the risk of intraoperative bleeds, which require massive blood transfusions and worsen prognosis. Recently, reports have been published on the use of rFVIIa prior to surgical procedures, which are likely to cause severe blood loss as well as for so-called emergency therapy of coagulation disorders during liver transplantation.

Application of recombinant activated factor VII for treatment of impaired haemostasis during liver transplantation in recipients with Wilson's disease--a report of two cases.

Recombinant activated factor VII (rFVIIa, NovoSeven, Novo Nordisk A/S, Denmark) is a treatment used to prevent and arrest intra- and postoperative bleeding in patients with haemophilia A or B complicated by circulating anticoagulants (inhibitors of FVIII and FIX) and in patients without haemophilia who spontaneously develop inhibitors of FVIII, i.e. in acquired haemophilia. Patients who qualify for liver transplantation due to liver dysfunction may have varying degrees of coagulation impairment and thus carry an elevated risk of massive bleeding and have worse prognosis. The authors administered recombinant activated factor VII to two patients with coagulation abnormalities in the course of Wilson's disease during liver transplantation.