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Acute liver failure (ALF) is a clinical syndrome associated with significant morbidity and mortality with a highly unpredictable outcome. We retrospectively analyzed 71 ALF patients (53 males; mean age = 27.5 ± 15.6 years) that underwent transjugular liver biopsy (TJLB) at our institution. The aims of this study are (i) to report our experience with TJLB in these patients, and (ii) to examine the role of liver histology in predicting their outcome. We also compared the histopathological findings between TJLB and explanted liver specimens in 31 patients who underwent liver transplantation (LT). Biopsy specimens were satisfactory for histopathological analyses in 69 (97.1%) patients, confirmed the clinical diagnosis in 56 (81.2%) patients, and altered the diagnosis in 13 (18.8%) patients. Minor complications were encountered in four (5.6%) patients. Percentage of hepatocyte necrosis was the only histological parameter that has significant discriminatory prognostic value, with no survivors having >75% necrosis without LT. In conclusions, TJLB is a safe technique for obtaining liver tissue in both adult and pediatric patients with ALF. Histological characteristics, mainly etiological diagnosis and degree of hepatocyte necrosis may assist in clinical decision-making for need of LT in these patients.

Peroral cholangioscopy with its limitations led to further research regarding development of SpyScope® technology. The aim of this retrospective study was to investigate the efficacy of a new device and the application of this device in our Liver Transplant Center.Charts of patients who had undergone evaluation with SpyScope® were retrospectively reviewed. Indications included pre-transplant as well as post-transplant evaluation of biliary strictures. If strictures or filling defects were noted by cholangiogram, SpyScope® was performed. Biopsy was obtained under direct visualization if necessary with SpyBite® biopsy forceps. Demographic features, indications for SpyScope® evaluation, results, and histopathological diagnoses were recorded.Ten patients (6 male, 4 female; median age: 55) had undergone SpyScope® procedure between August 2007 and January 2008. Six out of 10 cases were in the pre-transplant work-up period, referred to as Group I, while the remaining four were post-transplant patients, referred to as Group II. In Group I, 4 of 6 cases had undergone the procedure for work-up of primary sclerosing cholangitis prior to orthotopic liver transplantation. In Group II, indications were either strictures noted during the previous endoscopic retrograde cholangiopancreatography (n=2) or common bile duct stones with elevated total bilirubin levels and stones with long segment biliary stricture (n=2). In the patient with anastomotic stricture, the biliary lithiasis was eventually exposed just above the anastomotic stricture, after abundant lavage was applied at that level. All SpyBite® biopsy specimens were reported to be adequate samples for histopathological examination. No malignancy was detected among 4 patients with primary sclerosing cholangitis and patients with elevated CA 19-9.SpyScope® allows direct visualization of biliary strictures and SpyScope®/SpyBite® were found to be technically superior to conventional cholangiogram with better sampling than brushing obtained by endoscopic retrograde cholangiopancreatography. Prospective, multicenter, large volume studies are warranted to identify its sensitivity and specificity.

Currently available prognostic tools are inadequate to discern the molecular basis of the heterogenic response in hepatitis C virus (HCV)-infected patients treated with the current standard of therapy. The expression and biological function of immune mediators have been shown to be critical in all phases of the immune response to HCV infection and likely therefore influence host response. Herein, a biometric multiplex serum cytokine assay was utilized to characterize the immunomodulatory effects of host response in 10 HCV patients. Serum levels of 17 cytokines were compared before and after 1 month of treatment and against controls. Overall serum cytokine levels were significantly higher in patients (P < 0.05) than controls. Additionally, viral titers decreased in all patients after 1 month of therapy, as did overall serum cytokine levels in the cohort (P < 0.05). To assess relationships between changes in cytokine levels and changes in viral titer, the cohort was divided into three statistically distinct subgroups based on changes in viral titers. Specific sets of cytokines decreased in each group: decreases in CCL4, interleukin (IL)-2, CXCL8, and IL-1β correlated with the greatest drops in viral titer, decreases in IL-5, granulocyte colony stimulating factor (G-CSF), and CCL4 correlated with moderate drops in viral titer, and only CCL2 correlated with the lowest drops in viral titer. Interestingly, decreases in CCL4 levels correlated with decreases in viral titers in all patients. CCL4 controls leukocyte influx and thus propagates inflammation. In conclusion, these data raise the possibility that characteristic changes in host response modulate the therapeutic response, demonstrating the prognostic power of serum cytokine profiling in chronic HCV.

Background & Aims: There is growing evidence that the interplay of genetic susceptibility and environmental factors leads to primary biliary cirrhosis (PBC). In particular, family members of an infected individual have up to a 100-fold higher risk of developing PBC. Although concordant rates for identical twins in other autoimmune diseases range between 25% and 50%, there are no such data on PBC. Accordingly, we evaluated the concordance of PBC in a genetically defined population of twin sets and evaluated the clinical characteristics between concordant subjects. Methods: We identified 16 pairs of twins within a 1400-family cohort followed up by several centers worldwide, evaluated the diagnosis of PBC in all individuals, and determined the zygosity of sets reported as identical by the analysis of 2 highly variable HLA class II regions and 5 short tandem repeats. Results: Eight of 16 sets of twins were monozygotic. In 5 of 8 monozygotic twin sets, both individuals had PBC (pairwise concordance rate, 0.63). Among the dizygotic twins (n 8), no set was found to be concordant for PBC. Interestingly, the age at onset of disease was similar in 4 of 5 concordant sets of monozygotic pairs; however, there were differences in natural history and disease severity. Conclusions: The concordance rate of PBC in identical twins is among the highest reported in autoimmunity. However, discordant pairs were identified. The data show not only the role of genetics but also emphasize that either epigenetic factors and/or environment play a critical role.

Acute liver failure (ALF) is a clinical syndrome associated with significant morbidity and mortality with a highly unpredictable outcome. We retrospectively analyzed 71 ALF patients (53 males; mean age = 27.5 ± 15.6 years) that underwent transjugular liver biopsy (TJLB) at our institution. The aims of this study are (i) to report our experience with TJLB in these patients, and (ii) to examine the role of liver histology in predicting their outcome. We also compared the histopathological findings between TJLB and explanted liver specimens in 31 patients who underwent liver transplantation (LT). Biopsy specimens were satisfactory for histopathological analyses in 69 (97.1%) patients, confirmed the clinical diagnosis in 56 (81.2%) patients, and altered the diagnosis in 13 (18.8%) patients. Minor complications were encountered in four (5.6%) patients. Percentage of hepatocyte necrosis was the only histological parameter that has significant discriminatory prognostic value, with no survivors having75% necrosis without LT. In conclusions, TJLB is a safe technique for obtaining liver tissue in both adult and pediatric patients with ALF. Histological characteristics, mainly etiological diagnosis and degree of hepatocyte necrosis may assist in clinical decision-making for need of LT in these patients.

Chronic HBV infection is a dynamic state of interaction between HBV, hepatocytes, and the immune system of the host. A series of reactivation flares and remissions may occur due to multiple causes. Among them, spontaneous reactivation and immunosuppressive drugs including steroids or cancer chemotherapy are well known. This is due to immune-mediated destruction of HBV-expressing cells following withdrawal of immunosuppressive effect. Few cases have been reported in females during postpartum period. We report a case of fulminant hepatic failure during pregnancy in a previously unrecognized hepatitis B positive female requiring emergent liver transplantation.

Hepatic artery thrombosis (HAT) is the most frequent vascular complication following orthotopic liver transplantation. Urgent retransplantation has been considered as the mainstay therapy. Surgical revascularization is an effective alternative in asymptomatic patients. Endovascular therapies including intra-arterial thrombolysis, percutaneous transluminal angioplasty (PTA), and stent placement have shown encouraging results in recent years; however, their use remains controversial because of potential risk of hemorrhage. Until June 2009, 69 cases were published in 16 reports describing therapeutic potential of endovascular modalities. Interventions were performed as early as within 4 h to as late as 120 days in patients ranging from 4 months to 64 years of age. Majority of published reports suggested the use of urokinase. Thrombolysis was successful in 47 out of 69 (68%) patients. Bleeding was the most common complication including fatal intra-abdominal hemorrhage in three patients. Twenty-nine out of 47 (62%) patients underwent further intervention in the form of PTA, stenting, or both. The follow-up patency ranged from 1 month to 26 months. In conclusion, whenever possible, efforts should be made to rescue the liver grafts through urgent revascularization (surgical and/or endovascular) depending on patient's condition and interventional expertise at the transplant center; reserving the option of retransplantation for failure, complications, and cases with severe clinical symptoms or allograft dysfunction.

Ciliated foregut cysts of the liver are rare, with only 96 cases diagnosed since the first description in 1857. They are being increasingly diagnosed recently; the majority of the cases have been reported in the last 15 years. Although they bear a close resemblance to the simple cyst of the liver which has essentially a benign course, ciliated hepatic foregut cysts (CHFCs) can progress to malignancy with devastating consequences. It is imperative that this group of conditions be diagnosed and treated adequately.This review includes discussion of the data from all the 96 reported cases from English and non-English literature. Analysis of the incidence rates, embryogenesis, growth, clinical features, risk of malignancy and the prognosis are highlighted systematically. The roles of various diagnostic modalities including ultrasound, CT, MRI, fine needle aspiration cytology (FNAC), immunohistochemistry and surgery are further discussed.The mean age of patients with CHFC was 48+/-12 years. The male/female ratio was 1.1:1. The majority of patients with CHFC (62%) were asymptomatic, and the common mode of presentation was right upper abdominal pain. The cysts occurred in the left lobe in 51 patients, with sole location in segment IV in 44, and in the right lobe in 26. The average size of the cysts was 3.6+/-2.12 cm. The majority of the cysts were unilocular, and only 7 cases were multilocular. Cyst contents were described as viscous or mucinous in 73 patients, whereas bilious fluid was noted in 3. Large cysts having squamous carcinoma were cited in 3 patients, and 2 had extensive squamous metaplasia without malignancy. Others had benign histopathology.Clinicians have become increasingly aware of CHFC. Imaging alone is not diagnostic per se, but when considered in the context of the global picture does provide important clues to the diagnosis. FNAC is diagnostic by the presence of the ciliated columnar aspirate but lacks sensitivity. Infantile presentation is usually accompanied by biliary communication and mandates a different surgical approach. The demonstration of malignant transformation in 3 cases and its fatal course emphasizes the need for surgical resection in all cases once the diagnosis is made.

IgA deficiency is associated with high mortality (42% at 120 days) following liver transplantation (OLTx). Most of the mortality has been associated with enteric infections. Mother's milk, or human breast milk (HBM), is a rich source of IgA that is considered to have beneficial effects in terms of protection from microbial translocation and enteric infections. Two IgA-deficient OLTx recipients were given HBM orally for 10 days perioperatively. HBM was given in order to replenish intestinal IgA. Both patients had an excellent infection-free post-operative course. IgA levels in the serum rose from 5 to 10 mg/dl in one patient and from 7 to 30 mg/dl in the other. No complications from HBM administration were observed. We conclude that HBM can be used in IgA-deficient liver transplant recipients to reduce the risk of infectious complications in the post-operative period.

Utilization of endoscopy to both visualize and selectively biopsy an intestinal allograft has become the standard for early recognition and treatment of intestinal allograft rejection. Despite the widespread acceptance of the need for selective mucosal biopsies, it has not been shown that the histological features of intestinal allograft rejection are either localized or occur as part of a more diffuse phenomenon within a tubular allograft. To resolve these issues, 88 ileoscopies were performed in 12 small-bowel allograft recipients and mucosal biopsy samples were obtained at 5, 10, and 15 cm, respectively, from the ileal stoma. Each mucosal biopsy was labeled, processed, and evaluated individually for the presence and severity of any evidence for allograft rejection. The data obtained suggest that intestinal allograft rejection is a diffuse process, and biopsies obtained randomly from an ileal graft are likely to demonstrate evidence of allograft rejection when such is present.

Purpose The aim of this study was to determine serum prostate-specific antigen (PSA) levels in patients with liver cirrhosis. Patients and methods Between January 1995 and August 2001, 216 men with cirrhosis were evaluated. The extent of their liver disease was classified according to the Child-Pugh classification. Serum PSA levels were measured with the Hybritech Tandem-R RIA method and matched with age-related reference PSA levels. Digital rectal examination (DRE) was performed in all patients. Patients with elevated PSA levels and/or abnormal DRE were recommended to undergo further assessment including transrectal ultrasonography (TRUS) and biopsy performed by an urologist. Results Two hundred and sixteen men (mean age 54.09±9.09 years, range 25–76) with cirrhosis were examined. Their mean PSA value was 0.57±0.84 ng/mL and tended to be lower than in the normal population. The degree of PSA decrease was found to parallel the severity of the liver disease (p=0.002). The mean serum PSA level increased with each age decade in a statistically significant manner (pConclusion Serum PSA is influenced by the severity of liver disease and its levels tend to be lower in cirrhotic patients than in the normal population. However, serum PSA can still be considered a reliable marker in the clinical management of prostatic disease in patients with cirrhosis.

The effects of liver transplantation and the obligate use of cyclosporine on the subsequent clinical course of nine patients with sarcoidosis were examined. Two comparison groups, each consisting o...

Radiofrequency ablation for the treatment of advanced hepatocellular carcinoma- a single center's experience

Progress has been made in understanding skin diseases such as epidermolysis bullosa (EB). Epidermolysis bullosa is characterized by abnormal skin fragility and the formation of blisters and erosions of the skin and mucous membranes in response to minor trauma. The disease manifests in infancy, and, by childhood, patients have cutaneous scarring and mucosal involvement that can be severe. Treatment is palliative. Tacrolimus (FK506) is a new immunosuppressive agent used in organ transplantation. This agent has a number of nonimmune effects, one of which is augmentation of cell regeneration and repair.1 With the rationale of stimulating wound healing, we administered tacrolimus to a 5-year-old boy with dystrophic EB. There were no toxic effects, and scabs could form and affected areas heal since disease onset and treatment dramatically shortened the time to heal new lesions. Report of a Case. Our patient was normal at birth. Blistering began at 2 months; at 4