End-stage Primary Biliary Cirrhosis Research Articles

Usefulness of three-dimensional image navigation system for evaluation of hepatic artery before living donor liver transplantation: a case report

BackgroundThe evaluation of the hepatic vascular anatomy in living liver donors is increasingly being performed by three-dimensional (3D) computed tomography (CT) angiography. However, details of hepatic artery anatomy obtained by 3D CT angiography are not always superior to those obtained by angiography. Here, we report a case in which the 3D image navigation system helped to detect segment II, III, and IV arteries (A2, A3, and A4, respectively) that individually originated from the proper hepatic artery (PHA); this could not be detected by 3D CT angiography.Case presentationA 46-year-old man with end-stage primary biliary cirrhosis was admitted to our hospital for evaluation as a candidate for living donor liver transplantation. The patient’s younger sister, aged 43 years, was the only living donor candidate. The predicted left liver graft volume with the middle hepatic vein was found to be 403 mL using the region-growing method with 3D CT software. This volume was sufficiently large for the recipient because the standard liver volume of the recipient was 1095 mL. 3D CT angiography was performed twice but could not reveal the anatomical structure of the left and middle hepatic arteries. However, simulation using the region-growing method demonstrated individual branching off of A2, A3, and A4 from the PHA; conventional angiography demonstrated the same results. Each branch was approximately 1 mm in diameter, which was too small for secure anastomosis. Therefore, we selected the right liver graft for simplicity. The postoperative course of the donor and recipient was uneventful, and they were discharged on postoperative days 10 and 46, respectively.ConclusionsIn conclusion, reconstruction of the hepatic vasculature using the 3D software by region-growing method might be a useful adjunct for surgical planning in the evaluation of the hepatic arteries in living liver donors.

Exercise therapy in primary biliary cirrhosis: the importance of moving while sitting on a surgical waiting list—a case study

BackgroundIt is being increasingly recognised that reduced cardiorespiratory fitness is associated with poorer outcomes after major surgery. Exercise limitation and reduced aerobic capacity are common in people with end-stage liver...

The clinical analysis of orthotopic liver transplantation for primary biliary cirrhosis

To analyze the clinical outcome and complications of orthotopic liver transplantation (OLT) for patients with primary biliary cirrhosis(PBC). Clinical data of 25 patients with PBC who underwent OLT were analyzed retrospectively. The postoperative cumulative survival rate of the patients was calculated. The postoperative recurrence of PBC, de novo diseases and other complications were analyzed. A total of 25 patients were recruited including 22 females and 3 males. The average age was 49.1 years (range from 40 to 64 years). The score of model for end-stage liver disease (MELD) was 21.80±5.49, and the Mayo score 8.01±1.38. Four patients died after liver transplantation. The 1-year, 2-year and 7-year patient cumulative survival rates were 92.0%, 87.8% and 75.3%, respectively. Eight cases (32.0%) developed recurrent PBC based on histological evidence. The median time to recurrence was 17.5 months. One of the eight patients with recurrent PBC was diagnosed with de novo auto-immune hepatitis (AIH) simultaneously, who eventually died. Acute rejection and de novo hepatitis B virus (HBV) infection developed in thirteen (52.0%) patients and five (20.0%) patients, respectively. Two cases (8.0%) of de novo AIH were diagnosed, one of which was fatal. OLT is an effective procedure for end-stage PBC. Much attention needs to be paid to the post-transplantation complications, including recurrence of primary disease, combined de novo HBV infection, de novo AIH, etc.

In recurrent primary biliary cirrhosis after liver transplantation, biliary epithelial cells show increased expression of mitochondrial proteins.

In biliary epithelial lesions in primary biliary cirrhosis (PBC), mitochondrial proteins associated with deregulated autophagy are abnormally expressed. We examined whether this could be used as a diagnostic marker for end-stage PBC and recurrent PBC after liver transplantation. We examined the expression of the mitochondrial protein pyruvate dehydrogenase complex-E2 component and cytochrome c oxidase, subunit I (CCO), the autophagy-related marker microtubule-associated protein-light chain 3 (LC3), and p62/sequestosome-1 and the senescence markers p16(Ink4a) and p21(WAF1/Cip1) in small bile ducts and bile ductules in explanted livers from patients with PBC (n = 20) in comparison with liver tissue from control patients (n = 21) and post-transplant samples including recurrent PBC and cellular rejection (n = 28). Intense granular expression of mitochondrial proteins was significantly more frequent in small bile ducts in explanted livers with PBC than in control livers (p < 0.05). Post-transplant samples comprised of three groups: group A (positive for mitochondrial proteins, n = 7), group B (positive for either autophagy-related or senescence markers but negative for mitochondrial proteins, n = 7), and group C (all negative, n = 14). All but one case of group A were clinically and histologically diagnosed as recurrent PBC. In contrast, all cases of group B were diagnosed as cellular rejection. This study suggests that the expression of mitochondrial proteins in small bile ducts may be a useful diagnostic marker for end-stage PBC and recurrent PBC after liver transplantation.

Chronologic changes of explanted liver volume and the use of ursodeoxycholic acid in patients with end-stage primary biliary cirrhosis

The clinical presentation of Primary biliary cirrhosis (PBC) at the time of liver transplantation (LT) may have changed, due to the long-term use of ursodeoxycholic acid (UDCA). The aim of this retrospective study was to investigate whether the clinical characteristics of LT recipients with PBC have changed over the years. Of all 421 adults undergoing LT from 1997 to 2012 at our center, we included 85 recipients with PBC into the present study. The 85 recipients were divided into three groups according to the year LT was performed: group 1 (1997-2001, n = 29), group 2 (2002-2005, n = 29) and group 3 (2006-2012, n = 27). There were no significant differences in sex, recipient age, Model for End-Stage Liver Disease score, updated Mayo risk score for PBC, or liver-related complications except for esophageal varices among the three groups. Patients in group 1 were complicated with esophageal varices less frequently than those in the other two groups. In older cases, the ratio of explanted liver volume to standard liver volume (ELV/SLV) was significantly higher, and the duration of pre-LT UDCA treatment was significantly shorter. The duration of UDCA treatment was significantly correlated with ELV/SLV. Recent LT patients were characterized by more frequent portal hypertension and more severe liver atrophy, with longer UDCA therapy prior to LT, which might have prevented the somewhat rapid progression of liver failure characterized by hepatomegaly with insignificant fibrosis or portal hypertension.

Efficacy of Ursodeoxycholic Acid in Chinese Patients with End-Stage Primary Biliary Cirrhosis

AIM: To investigate the efficacy of ursodeoxycholic acid (UDCA) in Chinese patients with advanced primary biliary cirrhosis (PBC). METHODS: 55 PBC in-patients were treated with UDCA(1000mg/day) for 12 days(12.7±3.7 days). The biochemical response to UDCA treatment was evaluated before and after after treatment. RESULTS: Biochemical markers such as ALT, AST, ALP, GGT , TP, ALB, GLO,TBA, TBIL, DBIL were not improved significantly. During the therapy, no severe adverse effects leading to discontinuing the treatment were observed. CONCLUSIONS: UDCA is not effective for improving liver biochemical tests in patients with advanced PBC for short duration.

Regulation of dipeptidyl peptidase 8 and 9 expression in activated lymphocytes and injured liver

To investigate the expression of dipeptidyl peptidase (DPP) 8 and DPP9 in lymphocytes and various models of liver fibrosis. DPP8 and DPP9 expression were measured in mouse splenic CD4⁺ T-cells, CD8⁺ T-cells and B-cells (B220⁺), human lymphoma cell lines and mouse splenocytes stimulated with pokeweed mitogen (PWM) or lipopolysaccharide (LPS), and in dithiothreitol (DTT) and mitomycin-C treated Raji cells. DPP8 and DPP9 expression were measured in epidermal growth factor (EGF) treated Huh7 hepatoma cells, in fibrotic liver samples from mice treated with carbon tetrachloride (CCl₄) and from multidrug resistance gene 2 (Mdr2/Abcb4) gene knockout (gko) mice with biliary fibrosis, and in human end stage primary biliary cirrhosis (PBC). All three lymphocyte subsets expressed DPP8 and DPP9 mRNA. DPP8 and DPP9 expression were upregulated in both PWM and LPS stimulated mouse splenocytes and in both Jurkat T- and Raji B-cell lines. DPP8 and DPP9 were downregulated in DTT treated and upregulated in mitomycin-C treated Raji cells. DPP9-transfected Raji cells exhibited more annexin V⁺ cells and associated apoptosis. DPP8 and DPP9 mRNA were upregulated in CCl₄ induced fibrotic livers but not in the lymphocytes isolated from such livers, while DPP9 was upregulated in EGF stimulated Huh7 cells. In contrast, intrahepatic DPP8 and DPP9 mRNA expression levels were low in the Mdr2 gko mouse and in human PBC compared to non-diseased livers. These expression patterns point to biological roles for DPP8 and DPP9 in lymphocyte activation and apoptosis and in hepatocytes during liver disease pathogenesis.

Clinical significance of hepatectomy for primary biliary cirrhosis patients with hepatocellular carcinoma: Report of a single center case series and review of the published work

Hepatectomy for hepatocellular carcinoma (HCC) in patients with primary biliary cirrhosis (PBC) has seldom been reported, and the clinical significance of this procedure remains unclear, although HCC has often been observed in end-stage PBC patients. To understand the characteristics of hepatectomy on HCC in PBC patients, we examined seven cases at our institute, as well as 22 reported hepatectomy cases in the English-language and Japanese published work. Furthermore, to assess the treatment efficacy of hepatectomy for HCC in PBC patients, we compared these patients with viral hepatitis patients who underwent hepatectomies at our institute during the same period. In the review of 29 cases, more than 70% of the patients were aged over 65 years, and the mean Mayo risk score was low at 5.17. The resected tumors were mainly solitary (79%), and the median maximum tumor size was 37 mm. Approximately two-thirds of the patients met the Milan criteria. In the comparison between the PBC and viral hepatitis cases, there were no differences in the postoperative prognoses, although the tumor size was greater in the PBC cases. Hepatectomy for HCC in selected PBC cases is a feasible and potentially curative treatment option, similar to hepatectomy for HCC in viral hepatitis patients. This procedure is particularly useful for patients with preserved liver function who are not ideal candidates for liver transplantation.

The impact of liver transplantation on the phenotype of primary biliary cirrhosis patients in the UK-PBC cohort

Liver transplantation improves survival in end-stage primary biliary cirrhosis (PBC), but the benefit for systemic symptoms including fatigue is less clear. The aim of this study was to utilise the comprehensive UK-PBC Research Cohort, including 380 post-transplant patients and 2300 non-transplanted patients, to answer key questions regarding transplantation for PBC. Cross-sectional study of post-transplant PBC patients and case-matched non-transplanted patients. Detailed clinical information was collected, together with patient systemic symptom impact data using validated assessment tools. Over 25% of patients in the transplant cohort were grafted within 2 years of PBC diagnosis suggesting advanced disease at presentation. Transplanted patients were significantly younger at presentation than non-transplanted (mean 7 years) and >35% of all patients in the UK-PBC cohort who presented under 50 years had already undergone liver transplantation at the study censor point (>50% were treatment failures (post-transplant or unresponsive to UDCA)). Systemic symptom severity (fatigue and cognitive symptoms) was identical in female post-transplant patients and matched non-transplanted controls and unrelated to disease recurrence or immunosuppression type. In males, symptoms were worse in transplanted than in non-transplanted patients. Age at presentation is a major risk factor for progression to transplant (as well as UDCA non-response) in PBC. Although both confirmatory longitudinal studies, and studies utilising objective as well as subjective measures of function, are needed if we are to address the question definitively, we found no evidence of improved systemic symptoms after liver transplantation in PBC and patients should be advised accordingly. Consideration needs to be given to enhancing rehabilitation approaches to improve function and life quality after liver transplant for PBC.

Activation of FoxO3a/Bim axis in patients with Primary Biliary Cirrhosis

Impaired regulation of apoptosis has been suggested to play a role in the pathogenesis of Primary Biliary Cirrhosis (PBC). In this study, we analysed a signalling pathway that comprises the transcription factor FoxO3a and its downstream target Bim, a Bcl-2 interacting mediator of apoptosis. The tissues examined included livers explanted from patients with cirrhotic PBC, primary sclerosing cholangitis (PSC), alcoholic liver disease (ALD) and liver biopsies from patients with non-cirrhotic PBC. Large margin resections of hepatocellular carcinoma were used as controls. Expression of FoxO3a and Bim mRNA was significantly enhanced in both non-cirrhotic and end-stage PBC (2.2-fold and 4.3-fold increases, respectively), but not in the other disorders. Similarly, FOXO3a protein level was increased in end-stage PBC (P < 0.05 vs. control). A significant increase in Bim mRNA in non-cirrhotic and cirrhotic PBC was observed (2.2-fold and 8.2-fold respectively). In addition, the most pro-apoptotic isoform of Bim dominated in livers of PBC patients (2.5- fold increase vs. control; P < 0.05). Enhanced FoxO3a and Bim expression was associated with a substantial activation of caspase-3 in PBC (2-fold increase vs. controls; P < 0.0001), whereas it was decreased in both ALD and PSC (46% and 67% reductions respectively). The relationship between FoxO3a and Bim was further investigated in the livers of FoxO-deficient mice. The somatic deletion of FoxO3a caused a significant decrease in Bim, but not caspase-3 protein expression confirming the crucial role of FoxO3a in induction of Bim gene transcription. Our results imply that the FoxO3/Bim signalling pathway can be of importance in the livers of patients with PBC.

Proapoptotic protein bim and its upstream activator Foxo3A are overexpressed in primary biliary cirrhosis but not in primary sclerosing cholangitis

IntroductionApoptosis, with subsequent aberrant expression of pyruvate dehydrogenase complex (PDC-E2) on cell surface has been postulated to play an important role in the pathogenesis of Primary Biliary Cirrhosis (PBC). More...

A Rare Variation of the Biliary Tree of Relevance to Live Liver Donation

To the Editor: An appreciation of the potential anatomical variations of the vascular and biliary systems is essential for donor safety in the context of live donor hepatectomy (1, 2). We previously reported that variant biliary anatomy was present in a substantial proportion of donors with trifurcated portal veins (2, 3). The present report describes two rare cases of biliary anomaly with an independent bifurcation of a branch of segment 2 (B2) from the anterior or posterior bile duct. A 38-year-old male was admitted to our hospital to serve as a live donor for his sister, who had fulminant cryptogenic hepatic failure. Preoperative three-dimensional computed tomography (CT) of the donor did not demonstrate any anomalies of the portal vein, hepatic vein, or hepatic artery. Based on imaging and volume analysis of the liver, the donor underwent left hepatectomy with the caudate lobe. Intraoperative cholangiography demonstrated that B2 bifurcated independently from a branch of segment 7 (B7) (Figure 1A). Therefore, the left-lobe graft contained two bile duct orifices (B2 and B3). Intraoperative cholangiography confirmed that there was no bile leakage and that there was no biliary stenosis of the posterior branch. (A–C) Intraoperative cholangiography demonstrated that B2 independently bifurcated from B7 (case 1, A) or from the anterior branch (case 2, C). Magnetic resonance cholangiography demonstrated variant biliary anatomy, with B2 independently bifurcating from the anterior branch in case 2 (B). A 48-year-old male was admitted to our hospital to serve as a live donor for his wife, who had end-stage primary biliary cirrhosis. Preoperative three-dimensional CT of the donor demonstrated trifurcation of the portal vein, but there were no anomalies of the hepatic vein or hepatic artery. Magnetic resonance cholangiopancreatography (MRCP) demonstrated biliary anomaly with B2 bifurcating independently from an anterior branch (Figure 1B). Based on preoperative volume analysis, the left-lobe graft volume was considered to be too small for the recipient. Further, use of a right-lobe graft was complicated by the possibility that it possesses more than two bile duct orifices as well as two portal veins (anterior and posterior branches), which would increase the complexity of harvesting and of reconstruction. By contrast, the posterior segment graft had one bile duct orifice and portal vein with sufficient liver volume for the recipient. Therefore, the posterior segment graft was utilized for transplantation. The donor underwent posterior segmentectomy. Preoperative MRCP and intraoperative cholangiography demonstrated that B2 bifurcated from anterior branch (Figure 1C). The posterior segment graft had a single bile duct orifice and a single portal vein as well as a hepatic artery of the posterior branch and a right hepatic vein. Live donor liver transplantation requires careful graft selection to reduce the risk of complications. Biliary anomalies, which are sometimes associated with trifurcation of the portal vein, can complicate graft selection and donor surgery. This is the first report to describe independent B2 bifurcation from the anterior or posterior bile duct in a live donor. In donors with this anomaly, both the right and left-lobe grafts have multiple bile ducts. As illustrated in case 2 from the present report, the posterior graft represents a viable alternative for transplantation, provided graft volume is adequate. Preoperative evaluation of the biliary system has not been routinely utilized in our institution. Since dynamic CT may not detect important biliary variations, preoperative MRCP may be required to accurately characterize biliary anatomy, even in donor candidates without trifurcated portal veins. Based on the cost-benefit of this approach, we now routinely perform preoperative MRCP in all live donors in our institution. The authors of this manuscript have no conflicts of interest to disclose as described by the American Journal of Transplantation.

Successful liver transplantation from a living donor with asymptomatic pulmonary cryptococcosis

Successful liver transplantation from a living donor with asymptomatic pulmonary cryptococcosis

Sustained upregulation of sodium taurocholate cotransporting polypeptide and bile salt export pump and downregulation of cholesterol 7α-hydroxylase in the liver of patients with end-stage primary biliary cirrhosis

To examine the mRNA expression of hepatobiliary transporters in primary biliary cirrhosis (PBC) patients and to compare bile acid absorption, synthesis, and efflux in patients with non-end-stage and end-stage PBC, we obtained liver samples from PBC patients by percutaneous needle biopsy. End-stage PBC was defined as follows: histological stage IV; cirrhosis; serum total bilirubin, ≥4.0 mg/dl; and Child-Pugh Class C. The mRNA expression levels of sodium taurocholate cotransporting polypeptide (NTCP), bile salt export pump (BSEP), and hepatic cholesterol 7α-hydroxylase (CYP7A1) were significantly higher in the PBC patients than in the controls (P < 0.01). The mRNA levels of NTCP and BSEP were significantly higher in the end-stage PBC patients than in the controls (P < 0.01). However, hepatic CYP7A1 mRNA expression decreased significantly (by 70%) in the patients with end-stage PBC as compared to the controls and the patients with non-end-stage PBC (P < 0.01). The hepatic expression of transporters mediating bile acid influx and efflux showed sustained elevation, whereas that of the rate-limiting enzyme for bile acid biosynthesis was attenuated in the end-stage PBC patients. Thus, mechanisms may be present preventing the accumulation of toxic bile acids in the hepatocytes of end-stage PBC patients.

Cyclosporine A Protects Against Primary Biliary Cirrhosis Recurrence After Liver Transplantation

Primary biliary cirrhosis (PBC) reoccurs in a proportion of patients following liver transplantation (LT). The aims of our study were to evaluate the risk factors associated with PBC recurrence and determine whether recurrent disease constitutes a negative predictor for survival. One hundred and eight patients receiving LT for end-stage PBC were studied. Recurrent disease was diagnosed in 28 patients (26%). Probability of recurrent PBC at 5 years was 13% and 29% at 10 years with an overall incidence of 3.97 cases per 100 patient years. By univariate Cox analysis use of tacrolimus (HR 6.28, 95% CI, 2.44-16.11, p < 0.001) and mycophenolate mofetil (HR 5.21, 95% CI, 1.89-14.33, p = 0.001) were associated with higher risk of recurrence; whereas use of cyclosporine A (CsA) and azathioprine were associated with reduced risk of recurrence (HR 0.13, 95% CI 0.05-0.35, p < 0.001 and HR 0.27, 95% CI 0.11-0.64, p = 0.003, respectively). In the multivariate Cox analysis, only CsA was independently associated with protection against recurrence (HR 0.17, 95% CI 0.06-0.71, p = 0.02). Five-year probability of survival was 83% and 96%, in patients without and with recurrence (log-rank test, p = 0.3). Although PBC transplant recipients receiving CsA have a lower risk of disease recurrence, the development of recurrent PBC did not impact on long-term patient survival.

Favorable outcome ofde novohepatitis B infection after liver transplantation with lamivudine and adefovir therapy

De novo hepatitis B virus (HBV) infection after orthotopic liver transplantation (OLT) always progresses to chronic hepatitis because of the patients' immunocompromised status, and only a few then acquire hepatitis B surface antigen (HBsAg) seroconversion even with efficient antiviral therapy. Here we reported the case of a liver transplant recipient with de novo HBV infection who had a favorable outcome after lamivudine (LAM) and adefovir dipivoxil (ADV) antiviral therapy. The patient received OLT because of end-stage primary biliary cirrhosis and was found to have de novo HBV infection 3 months later. She was treated with LAM, and her serum HBV DNA turned undetectable 2 weeks later. However, serum HBV DNA turned detectable again after 9 months of LAM therapy and a YMDD mutation was detected. The addition of ADV was efficient to treat LAM-resistant HBV. After 3 months of combination therapy, LAM was stopped and ADV monotherapy was continued. HBsAg seroconversion was achieved after an additional 12 months. The prevention and treatment of de novo HBV infection after OLT is discussed.

Globus pallidus magnetization transfer ratio, T1 and T2 in primary biliary cirrhosis: Relationship with disease stage and age

To determine whether the magnetization transfer ratio (MTR) of the globus pallidus (GP) in patients with primary biliary cirrhosis (PBC) correlates with age, disease stage, and fatigue, using T(1) and T(2) mapping to determine whether the mechanism of change is consistent with manganese deposition in the GP as suggested by previous reports. In all, 30 early-stage PBC patients, four end-stage PBC patients, and 14 female controls were recruited to age-matched groups. MTR, T(1) and T(2) measurements were performed. A bilateral region of interest (ROI)-based analysis was used to calculate GP MTR, T(1), and T(2) values. These were correlated with age, disease status, and fatigue. MTR measurements showed a significant, negative correlation with age for controls and early-stage PBC patients, a positive correlation with T(2), and no correlation with T(1). Only GP T(2) is significantly lower in early-stage PBC patients than controls, while end-stage patients demonstrated a simultaneous reduction in T(1) and MTR, consistent with GP manganese deposition. MTR measurements correlate with age in both early-stage patient and control groups, but are not associated with manganese deposition or fatigue severity: only the end-stage disease group shows changes in MTR, T(1), T(2) that are consistent with manganese deposition.

Artificial Vascular Graft for Inferior Vena Cava Reconstruction in Living Donor Liver Transplantation: A Case Report

Artificial grafts are not recommended because of the high incidence of thrombogenic effects. However, in some situations, such as emergency or when no vascular bank is available, an artificial graft must be used. We present a case in which a polytetrafluoroethyline graft was used as a conduit to reconstruct the retrohepatic vena cava severed during living donor liver transplantation (LDLT). A 48-year-old woman had end-stage primary biliary cirrhosis for 5 years received a right lobe liver graft from her son. The retrohepatic vena cava was divided and ligated in several sequences. The upper end of the severed retrohepatic vena cava retracted into the liver parenchyma. The lower end of the severed vena cava was distended, with multiple stitches. A 16-mm artificial graft was used as a conduit to replace the inferin vena cava for outflow reconstruction. The patient tolerated the complicated procedure well. No anticoagulant was used throughout the entire course. The patient has been well with excellent liver function after follow-up for more than 5 years. Magnetic resonance imaging and Doppler ultrasonographic studies showed good patency of the cava with no evidence of thrombosis. We suggest use of an artificial graft in living donor liver transplantation, in particular in urgent situations when autologous or allogeneic vessels are not available.

Serum sterols in patients with primary biliary cirrhosis and acute liver failure before and after liver transplantation

Liver diseases modify sterol metabolism. Liver transplantation (LTX) provides a model to evaluate the impact of disease-affected liver on sterol metabolism. We studied serum sterol profiles and their relationships to other biochemical markers in consecutive cholestatic patients with acute liver failure (ALF, n=39) and end-stage primary biliary cirrhosis (PBC, n=67) before and 27d after LTX. Accordingly, we determined serum levels of sterols, bilirubin and prealbumin. Due to weak cholesterol synthesis of ALF-patients before LTX, their serum levels of cholesterol, lathosterol/cholesterol, cholestanol/cholesterol and lathosterol/campesterol were 18%-41% lower (P<0.05 for each) than in PBC, but ratios of phytosterols to cholesterol were equal. In general, non-cholesterol sterol ratios reflected bilirubin and prealbumin concentrations. Interrelation of surrogate sterols showed that homeostasis of cholesterol metabolism prevailed in lowest cholestanol tertile of ALF-patients consistently, but not in PBC. After LTX, cholesterol levels and lathosterol ratios increased in both groups and phytosterol ratios decreased (P<0.01). Cholestanol decreased profoundly in PBC, but remained 26% higher than in ALF (P<0.05). Homeostasis of cholesterol metabolism was maintained only in ALF. Metabolism of phytosterols was equal in study groups. PBC- and ALF-patients have differential patterns in their serum sterols and cholesterol metabolism.

Persistent pyrexia post-liver transplantation: A unique case of pericardial tuberculosis

A 60-year-old Caucasian woman was admitted into the hospital with a two-week history of dry cough, fever and breathlessness. There were no other complaints. She had an orthotopic liver transplant (OLT) three months previously for end-stage primary biliary cirrhosis (PBC) and was on azathioprine 125 mg and tacrolimus 9 mg per day. On examination, she was pale, tachycardic and temperature was 38.7 °C. There was medium to coarse crepitations in the left lung base. Initial investigations were: white cell count (WCC) 8.5 (NR 4–11×10/L), neutrophils 4.5 (NR: 2–7.5×10/L), lymphocytes 1.5 (NR 1.5– 4×10/L), haemoglobin (Hb) 7.1 (NR: 11–16 g/dL), platelets 375 (NR: 150–400×10/L), C-reactive protein (CRP) was 182 (NR: b10 mg/L), AST 86 (NR: 5–43 U/L), ALP 965 (NR: 70– 330 U/L), bilirubin 12 (NR:1–22 μmol/L) and creatinine 133 (NR: 50–111 μmol/L). Chest radiograph (CXR) showed clear lung fields but increased cardio-thoracic ratio (C: T=0.6). Blood, urine, sputum and stool cultureswere negative. Shewas started on intravenous tazocin (piperacillin–tazobactam) 4.5 g t.d.s. for a possible lower respiratory tract infection. Despite completing antibiotics, she remained unwell with swinging pyrexia (37.5 to 39 °C). Abdominal ultrasound scan was normal but a trans-thoracic echocardiogram confirmed loculated pericardial effusion. Pericardiocentesis provided symptomatic relief; pericardial fluid was sent for microscopy, culture and cytology. All were negative, including a Ziehl– Neilsen (ZN) stain. Peripheral blood cytomegalovirus (CMV) polymerase chain reaction (PCR) showed high viral loads at 1.7×10 copies/mL.