These were age at infection, patient gender, HCV genotype, the presence of steatosis, BMI, and inflammatory grading. An interaction term was specified between steatosis and viral genotype, to reflect that the effect of steatosis on fibrosis progression may be specific for different genotypes. The final model was chosen on the basis of the minimum Akaike information criterion. The results summarized in Table 3 show that age at infection was the variable most strongly and positively associated with

fibrosis progression RAD001 in vivo (P < 2E-16). Male gender (P = 0.012), HCV genotype 3 (P = 7.19E-04), and the presence of steatosis (P = 0.012) also resulted in being significantly associated with an accelerated rate of fibrosis progression. Importantly, the two IL28B SNPs CHIR-99021 solubility dmso had no significant effect on the outcome, and thus they were removed from the final model (Table 3). It is

worth pointing out that HCV genotype 2 was associated with a slower rate of fibrosis progression (P = 0.034), as also suggested in a previous study.14 Single-term deletion analysis further confirmed the role of these explanatory variables. Figure 1 shows the remarkable effect of age at infection on rate of fibrosis progression. In contrast, no effect could be attributable to the host IL28B genotype. Notably, no impact on FPR was found, also assuming a dominant model of inheritance for the alleles, rs8099917 G or rs12979860 T, previously associated with treatment failure (data not shown). On the contrary, when these two SNPs were tested for association with therapy outcome, using data from 91 patients with HCV genotype 1 and available treatment information, the effect was readily detectable (see Supporting Information). Actually, rs12979860 genotypes CT and TT versus CC resulted in being associated

with treatment failure (P < 0.01, odds ratio = 3.6, 95% CI = 1.3-10.2), in agreement with previous reports. Finally, we evaluated the role of explanatory variables, excluding individuals that acquired Rebamipide the infection at birth, to exclude possible confounding factors originating from the inclusion of a group of patients that acquired the infection in the setting of an immature immune system. In this subgroup of patients, we obtained the same estimates of the parameters and confirmed the lack of any effect by the two IL28B polymorphisms and the significant role of the nongenetic factors (Supporting Table 1). In summary, the model outlined in Table 3—which includes patient gender, age at infection, viral genotype, and steatosis—explained an estimated 34% of the phenotypic variability, showing that patient age at infection has a major, highly significant role on fibrosis progression, in agreement with other reports.14-16 The estimated effect on fibrosis progression of each additional year at infection was a 2.8% (95% CI = 2.2%-3.4%) increase in FPR.

6 and 4.1 months in the sorafenib- and placebo-treated patients (hazard ratio = 0.75; 95% CI, 0.54–1.05). However, a wide difference was seen in the overall survival according to the presence or absence of MVI and/or EHS in both trials. Among the sorafenib-treated patients in the SHARP trial, median overall survival was 14.5 months for patients without MVI and/or EHS versus 8.9 months for patients with MVI and/or EHS. In the Asia–Pacific trial, median overall survival was 14.3 and FLT3 inhibitor 5.6 months, respectively. Therefore, the survival benefit of sorafenib monotherapy in advanced HCC patients with PVT or extrahepatic metastasis is still not

satisfactory, especially in Asian patients. To overcome these limited therapeutic responses, the development of more effective therapy is urgently needed. When monotherapy is not enough to control tumor progression, combined therapy or a multidisciplinary DAPT research buy approach can be considered. In our previous study, localized concurrent intra-arterial

chemoradiation therapy plus repeated HAIC combination treatment achieved favorable results in patients with localized advanced HCC with PVT.21 The efficacy of sorafenib in advanced HCC has been examined in combination with conventional systemic chemotherapy, such as doxorubicin, tegafur/uracil, 5-FU, and mitomycin.22 In spite of the positive results in combining sorafenib with chemotherapeutic agents, these clinical trials were non-randomized and conducted on a small number of patients. Combining other agents with different mechanisms of action is theoretically a reasonable therapeutic approach in the treatment of advanced HCC. Also, combining molecularly-targeted therapies that block different pathways compared with sorafenib monotherapy is an attractive approach. Non-specific serine/threonine protein kinase Future studies should evaluate the efficacy and safety of combination therapy of sorafenib plus conventional chemotherapeutic

agents or other targeted agents. Moreover, the use of sorafenib in combination with other therapeutic options, such as transcatheter arterial chemoembolization, internal, or external radiotherapy in advanced HCC is also a topic of interest, and further studies on this topic are warranted. In conclusion, the management of patients with advanced HCC with conventional cytotoxic systemic monotherapy or combined therapy has not been satisfactory over the last decades. At present, sorafenib is the only approved therapy for advanced HCC. However, to improve the survival benefit of sorafenib by maximizing the therapeutic efficacy, a major challenge is how to refine treatment strategies and select proper patients. In addition, we should consider not only therapeutic efficacy, but also practical issues, such as medical cost in Asia. Based on these efforts, it is expected that advanced HCC will be considered a treatable and increasingly curable disease in the near future.

1A,B). Quiescent, ramified microglia cells continuously monitor their surrounding environment through filopodia expansion and retraction.19 As shown by time-lapse microscopy, primary microglia in culture expresses fine-structured filopodial processes which continuously reorganize while probing their microenvironment (Fig. 1C). However, upon addition of NH4Cl (5 mmol/L) the cells retract within seconds their filopodia to a length of about 50% of that found in untreated control cells (Fig. 1D). This retraction was accompanied by a reduction of the cell diameter by about 30% as compared

with the control condition (Fig. Pifithrin�� 1D). Microglia are a major phagocytosing cell type that removes cell debris and pathogens in the brain.15, 16 Brain dysfunction in neurodegenerative diseases is frequently associated with increased phagocytotic activity, EPZ 6438 which may represent another surrogate marker for microglia activation.15, 16, 19 As shown in Fig. 2, ammonia inhibited phagocytosis in a subset of microglial cells, thereby reducing overall phagocytosis to

approximately 65% of the control condition (Fig. 2A). However, in phagocytosing microglia cells, the number of phagocytosed fluorescent latex beads per cell was not significantly affected (Fig. 2B,C). These findings may reflect the known heterogeneity of microglia in the brain. Upon activation, microglia increase the expression of the ionized calcium-binding adaptor molecule-1 (Iba-1).18 This protein transduces calcium signals for reorganization of the cytoskeleton, thereby allowing for morphology changes and cell migration.18 As shown by immunofluorescence analysis (Fig. 3A), NH4Cl induced Iba-1 up-regulation in cultured microglia in a time- and triclocarban concentration-dependent manner (Fig. 3A-D). Significant Iba-1 up-regulation occurred 6 hours after NH4Cl (5 mmol/L) treatment (Fig. 3A) but not at 1 or 3 hours of exposure (data not shown). Ammonia concentrations of 5 mmol/L (6 hours) and 1 mmol/L (20 hours), respectively, were sufficient to induce a significant Iba-1 up-regulation

to approximately 1.25 or 1.5-fold of control. However, Iba-1 messenger RNA (mRNA) expression was not up-regulated by NH4Cl (5 mmol/L) after 6 hours (0.65 ± 0.12-fold of control, n = 3) or 20 hours after treatment (0.70 ± 0.07-fold of control, n=4 [P = 0.02]). Microglia activation is frequently accompanied by an increased formation of ROS and an induction of iNOS.13, 14, 16 As shown in Table 1, NH4Cl increased microglial ROS production significantly in a time- and dose-dependent manner as measured by DCF-fluorescence. Pretreatment of microglia with apocynine (300 μmol/L, 30 minutes pretreatment) completely abolished the NH4Cl (5 mmol/L, 6 hours)-induced DCF-fluorescence increase suggestive for an activation of the reduced form of nicotinamide adenine dinucleotide phosphate (NADPH)-oxidase by ammonia (0.96 ± 0.05-fold of apocynine-treated controls, n = 4).

Z. aplanosporum also possessed a combination of vegetative and reproductive features characteristic

of Zygogonium, such as presence of short branches, rhizoidal outgrowths, thickened vegetative cell walls, purple-colored cell content, small compressed-globular chloroplasts as well as predominant asexual reproduction. Z. aplanosporum and Z. californicum were deeply embedded in a larger clade of Zygnema both in rbcL and cox3 analyses. Based on our observations, there are no features or combination of features that separate Zygnema and Zygogonium. Therefore, we conclude that Zygogonium is probably a synonym of Zygnema. “
“Sexual reproduction is documented for the first time in field populations of the pennate diatoms Pseudo-nitzschia australis Freng. and P. pungens (Grunow Selleckchem Hydroxychloroquine ex Cleve) Hasle (var. cingulata Villac and EPZ-6438 price hybrids between var. cingulata and var. pungens). A bloom dominated by these species began on June 26, 2006, along Kalaloch Beach, Washington, USA, coincident with a drop in the Si(OH)4:NO3 ratio

to below two. Multimodal size distributions were detected for both species, and synchronous auxosporulation occurred within the smallest size class during a 3-week window. Auxospores and initial cells created a new class of large cells, and cells in the intermediate size classes increased in abundance during auxosporulation. Mating cells of both species were attached to colonies of surf-zone diatoms. Paired gametangia, gametes, zygotes, auxospores, and large initial cells were found. C1GALT1 Auxosporulation began first for P. pungens (June 30), apparently once a critical, high cell concentration was reached, followed by P. australis (July 5), when the total Pseudo-nitzschia cell concentration reached 929,000 cells · L−1. Low frequencies of auxosporulation occurred throughout the bloom but increased 4-fold for P. australis and 3-fold for P. pungens when macronutrients were reduced to low levels on July 11.

A 2-year life cycle was estimated for P. australis and 3 years for P. pungens, both with annual auxosporulation. Domoic acid (DA) in razor clams reached a maximum of 38 μg DA · g−1 on July 18. A significant relationship existed between the percent of cells within the new size range and DA concentrations in razor clams on the same beach. “
“Primary productivity by plants and algae is the fundamental source of energy in virtually all food webs. Furthermore, photosynthetic organisms are the sole source for ω-3 and ω-6 essential fatty acids (EFA) to upper trophic levels. Because animals cannot synthesize EFA, these molecules may be useful as trophic markers for tracking sources of primary production through food webs if different primary producer groups have different EFA signatures.

Prion diseases are transmissible experimentally and naturally, selleck compound and enormous

efforts have been directed towards establishing the nature of the transmissible agent. Stanley Prusiner proposed the prion hypothesis in 1982, suggesting that the transmissible agent was composed entirely of a modified host protein, the prion protein (PrP), which was partially resistant to proteolytic degradation, with no nucleic acid component [3]. The normal form of the prion protein (PrPc) is expressed at the highest levels in neurones within the brain [2]. In prion diseases, an abnormal isoform of PrP (designated PrPSc) accumulates in the brain, with an identical amino acid sequence to PrPc, but an increased beta-sheet content that renders it relatively resistant to proteolytic digestion [2]. PrPSc is (at least) the main constituent of the transmissible agent and is closely associated with infectivity. The high beta sheet

content of PrPSc confers stability; conventional means of bacterial and viral decontamination are generally ineffective for prions. None of the current measures recommended for decontamination of prions is guaranteed to remove all infectivity [4], and these measures are not applicable to learn more blood or plasma products. The commonest human prion disease is sporadic Creutzfeldt–Jakob disease (CJD), which has an incidence of around 1.5 per million of the population, with a worldwide distribution [5]. The causes of sporadic CJD are unknown, but there is evidence of a genetic predisposition. In the human prion protein gene (PRNP) located on chromosome 20, there is a naturally occurring polymorphism at codon 129, which can encode either methionine or valine (Table 2) [6]. In contrast to the normal Caucasian population, there is a predominance of homozygotes in sporadic CJD, particularly methionine homozygotes (Table 2). Surveillance of CJD was

recommenced in the Pazopanib chemical structure United Kingdom (UK) in 1990, to identify any possible effects of BSE. Over 180 000 clinical cases of BSE have been identified in the UK since the 1980s, but when allowances are made for asymptomatic infections, it has been estimated that up 3 million infected cattle may have entered the UK human food chain [7]. In 1996, the National CJD Surveillance Unit reported a new form of human prion disease in the UK, now known as variant CJD [8]. Variant CJD has a clinical and pathological phenotype that is distinct from sporadic CJD [9]. All probable and definite variant CJD patients who have undergone genetic testing are methionine homozygotes at the codon 129 polymorphism of the PRNP gene, indicating susceptibility to variant CJD in this genetic subset. However, a recent possible case of variant CJD has been reported in a heterozygote (methionine/valine) at this genetic locus [10].

However, further follow-up are required for the evaluation of efficacy between

two groups. Disclosures: The following people have nothing to disclose: Sangheun Lee, Jun Yong Park, Hana Park, Moon Young Kim, Sang Hoon Ahn, Pumsoo Kim, Kwang-Hyub Han BACKGROUND: Serum HBsAg levels can be used as a surrogate marker for the interaction between the immune system and the hepatitis B virus. We aimed to study the kinetics of HBsAg levels in chronic hepatitis B (CHB) patients who discontinued nucleos(t)ide analogue (NA) therapy. METHODS: We included 94 consecutive patients who stopped NA after at least one year of therapy. Patients could be HBeAg-positive or HBeAg-negative at start-of-therapy, but all were beta-catenin inhibitor HBeAg-negative and had undetectable HBV DNA at time of discontinuation. Relapse was defined as HBV DNA >2,000 IU/mL measured twice 6 months apart within one year, or retreatment after an initial HBV DNA increase. RESULTS: In total, HBsAg decline could be calculated for 69 patients, of whom 26 were relapsers. The on-treatment HBsAg decline was

comparable between relapsers and sustained responders, whereas the post-treatment HBsAg decline was greater in sustained responders compared to relapsers (Figure). After adjustment for start-of-therapy HBeAg-status, cirrhosis, and consolidation therapy duration, post-treatment HBsAg decline remained significantly Selleck 5-Fluoracil associated with absence of relapse (p=0.014). Forty-seven patients had paired on-treatment and post-treatment HBsAg decline data available. Within Methane monooxygenase this group, HBsAg decline increased in sustained responders from −0.09 to −0.17 log IU/mL per year (p=0.009) after NA therapy

cessation, but not in relapsers (−0.03 vs. −0.07 log IU/ mL per year; p=0.85). CONCLUSIONS: After stopping long-term NA therapy in CHB patients, HBsAg decline increased in sustained responders, whereas these levels remained at the same level in relapsers. These results suggest the potential of host-induced immune control and viral clearance in sustained responders after NA therapy cessation. Disclosures: Colina Yim – Advisory Committees or Review Panels: Merck Canada, Gilead, Janssen Jordan J. Feld – Advisory Committees or Review Panels: Idenix, Merck, Janssen, Gilead, AbbVie, Merck, Theravance, Bristol Meiers Squibb; Grant/Research Support: AbbVie, Boehringer Ingelheim, Janssen, Gilead, Merck Robert J. de Knegt – Advisory Committees or Review Panels: MSD, Roche, Norgine, Janssen Cilag; Grant/Research Support: Gilead, MSD, Roche, Janssen Cilag, BMS; Speaking and Teaching: Gilead, MSD, Roche, Janssen Cilag David K. Wong – Grant/Research Support: Gilead, BMS, Vertex, BI Harry L.

The PD-332991 only production of TNF-α in the liver is thought to be from hepatic macrophages known as Kupffer cells, predominantly in response to bacterial lipopolysaccharide (LPS). Primary cultured rat hepatocytes were used to analyze TNF-α expression in response to the pro-inflammatory cytokine, interleukin-1β (IL-1β). Livers of rats subjected to LPS-induced endotoxemia were analyzed. Immunocytochemistry and enzyme-linked immunosorbent assays demonstrated

that IL-1β-treated rat hepatocytes secreted TNF-α, and RNA analyses indicated that TNF-α mRNA was induced specifically by IL-1β. Northern blot analysis showed that not only mRNA, but also a natural antisense transcript (asRNA), was transcribed from the rat Tnf gene in IL-1β-treated hepatocytes. TNF-α was detected in the hepatocytes of LPS-treated rats. Both TNF-α mRNA and asRNA were expressed in the hepatocytes of LPS-treated rats, human hepatocellular carcinoma and human monocyte/macrophage cells. To disrupt the interaction between TNF-α asRNA and TNF-α mRNA, sense oligonucleotides corresponding to TNF-α mRNA were introduced into rat hepatocytes resulting in

significantly increased levels of TNF-α mRNA. One of these sense oligonucleotides increased a half-life of TNF-α mRNA, suggesting that the TNF-α asRNA may reduce the stability of TNF-α mRNA. BTK inhibitor mouse IL-1β-stimulated rat hepatocytes are a newly identified source of TNF-α in the liver. TNF-α mRNA and asRNA are expressed in rats and humans, and the TNF-α asRNA reduces the stability of the TNF-α mRNA. Hepatocytes and TNF-α asRNA may be therapeutic targets to regulate levels of TNF-α mRNA. “
“Although coffee consumption has been associated with reduced frequency of liver disease, it is unclear whether the effect is from coffee or caffeine and whether there is an effect on hepatic fibrosis specifically.

This study was undertaken to use a food-frequency instrument for dietary caffeine consumption to evaluate the relationship between caffeine intake and liver fibrosis. Patients undergoing liver biopsy completed a detailed caffeine questionnaire on three occasions over a 6-month period. Caffeine intake was compared between patients with mild and advanced liver fibrosis (bridging fibrosis/cirrhosis). Logistic regression was used MG-132 ic50 to evaluate the association between caffeine consumption and hepatic fibrosis. One hundred seventy-seven patients (99 male, 104 white, 121 with chronic hepatitis C virus [HCV] infection) undergoing liver biopsy completed the caffeine questionnaire on up to three occasions. Results from repeated questionnaires were consistent. Daily caffeine consumption above the 75th percentile for the cohort (308 mg = approximately 2.25 cups of coffee equivalents) was associated with reduced liver fibrosis (odds ratio [OR], 0.33; 95% confidence interval [CI], 0.14-0.80; P = 0.

Necrosectomy could be successfully performed through the stent in all needed cases. The stent was easily extracted at the end of the therapy period. Based on results of this study, stent migration rate was low. The Nagi stent™ may be considered as the first option for patients undergoing EUTMD of PFC’s. Further prospective randomized

controlled trials comparing this stent to multiple plastic stents is recommended. Key Word(s): 1. pseudocyst; 2. WOPN; 3. endoscopic drainage; 4. fully covered SEMS; Details N (total = 21) Percent Puncture site Esophagus 1 5 Stomach 18 86 Duodenum 2 9 Access – 19G FNA needle 21 100 Balloon dilatation of track 4 mm 9 43 6 mm 3 14 8 mm 8 38 15 mm 1 5 Concurrent drainage Double pigtail plastic stent 10 48 Nasocystic drain 7 33 Necrosectomy 7 33 Stent removal   14 67 Days after insertion, mean (range) Pembrolizumab 49.1 (45–60) Technical success 21 100 Clinical success 21 100 Complications – stent migration 1 5 Presenting Author: VINITA CHAUDHARY Additional Authors: SURINDERS RANA, DEEPAKK BHASIN, CHALAPATHI RAO, RAJESH GUPTA Corresponding Author: DEEPAKK BHASIN Affiliations: PGIMER Objective: Pancreatic pseudocysts are usually located in peripancreatic area and are rarely located at atypical locations. There is paucity of data on EUS features of pseudocysts at atypical locations.

Methods: Retrospective analysis of patients with pseudocysts at atypical locations seen over last four years. Results: Ten patients (all males; age 21–58 years) were studied. The location of pseudocysts buy CP-690550 was: mediastinum (6), liver (1), and

intramural gastric wall (1) and duodenal wall (2). The pseudocysts occurred as a consequence of acute pancreatitis in 2 patients (alcohol in both) and chronic pancreatitis in 8 patients (alcohol in all and one of these patient had coexistent pancreas divisum). All patients presented with abdominal pain. One each patient also had dysphagia, gastric outlet obstruction and jaundice because of biliary obstruction. The pseduocysts were well demonstrated on EUS. It could also identify necrotic debris as echogenic contents in the cyst and 9/10 (90%) STK38 of patients did not have any necrotic debris in the pseudocysts. In patients with intramural pseudocysts, EUS could clearly demonstrate its intramural location (in all the three patients muscularis propria could be seen intact around the pseudocyst). All the three patients of intramural pseudocyst were successfully treated with single time EUS guided aspiration whereas patient with intra hepatic pseudocyst was successfully manage conservatively. Five of 6 patients with mediastinal pseudocyst were successfully treated with endoscopic transpapillary drainage whereas one patient refused further treatment and was lost to follow up. Conclusion: EUS is a useful investigation for pancreatic pseudocysts at atypical locations. Key Word(s): 1. EUS; 2. pseudocyst; 3. pancreatitis; 4.

22% were classical SBP, 72% were CNNA and 6% were MNB. E. coli was the commonest organism isolated; all strains of it were resistant to third generation cephalosporins whereas 78% were resistant to quinolones.E. coli isolates were sensitive to imipenem, but only

67% were sensitive to a combination of third generation cephalosporin and beta lactamase inhibitor. Conclusion: SBP is common in patients with CL with ascites and is mostly caused by E coli. A high percentage of E coli are resistant to cephalosporins and quinolones, but sensitive to imipenem or a combination of 3rd generation cephalosporin VX-770 cost and beta lactamase inhibitor. Key Word(s): 1. SBP; 2. cirrhosis; 3. ascites; 4. antibiotic ; Presenting Author: LAURA MASALAITE Additional Authors: JONAS VALANTINAS, JUOZAS STANAITIS Corresponding Author: LAURA MASALAITE Affiliations: Clinic of gastroenterology, nephrourology and surgery, Medical Faculty, Vilnius University, Objective: Endoscopic band ligation (EBL) has a high tendency of variceal recurrence. The aim was to evaluate the value of esophageal collateral veins as predictors for variceal recurrence after EBL. Methods: 31 patient with large esophageal varices and EBL indicated were enrolled in prospective

TAM Receptor inhibitor study. Endosonography was performed before EBL and collateral CYTH4 veins were classified into three types (peri-esophageal

(peri-ECV), para-esophageal (para-ECV) and perforating veins) and two grades (mild and severe). Varices were ligated every 2 weeks until obliteration and upper endoscopy was performed every 3 month to detect any form of varices or red-color signs. Relationship between endosonography findings and the variceal recurrence rate was analysed (p value < 0,05 was considered statistically significant). Results: Variceal recurrence was detected in 5 patients (16,1%) within 3 months, in 8 patients (36,6%) within 6 months and in 12 patients (75%) within 12 month. 16 of the 31 patients were followed for 12 month and were divided into non-recurrence and recurrence (early and late) groups. No significant difference between these groups regarding collateral veins type and grade was found (table 1 and 2). Mathematical cox proportional hazards model of data found that severe peri-ECV are associatedwith higher and earlier recurrence risk after EBL (hazard ratio 1,57). Conclusion: Our study showed that endosonography findings does not predict variceal recurrence after EBL. Our results may be conflicting due to a small sample size and short follow up period. Key Word(s): 1. Esophageal varices; 2. Ligation; 3. Variceal recurrence; 4.

All data were placed in a database with names of patients and other identifying information removed for confidentiality to the extent permitted by law. Institutional MAPK inhibitor Review Board approval was obtained prior to study commencement. Statistical analysis of comparisons between laboratory data among both subject and control patients was performed using unpaired t tests. Pathology findings in the 10 biopsy specimens from all prospectively identified minimal change cases are shown in Table 1. A retrospective chart review

was then conducted of the 10 prospectively identified subject patients and six were identified who had retrievable clinical data. All 10 PBC control patients had retrievable clinical data. The average length of follow-up was 2 years. Baseline characteristics and clinical data on the subject and PBC control patients are summarized in Tables 2-5, respectively. There were no statistically significant differences between baseline characteristics or laboratory values before and after treatment, among both sets of patients using paired t-test analysis. In addition, total bilirubin levels (not presented in tables) among both sets of patients were within normal limits with no statistically significant differences PD-0332991 manufacturer before or after treatment.

No exposures to known hepatotoxins (prescription or non-prescription) were identified in the patients upon chart review. Study patients had an age distribution of 52 ± 7 years; PBC control patients had an age distribution of 52 ± 12. All suspected or diagnosed PBC patients were female. Clinical data for the CHC patients

showed a male:female gender distribution of 5:6 and age distribution of 48 ± 9 years. These age differences are not statistically significant. Patient 1 presented initially with symptoms of fatigue and pruritus. On laboratory evaluation the patient’s AP and gamma-glutamyl transpeptidase (GGT) levels were found to be elevated for at least 1 year. The patient also had a positive AMA, as well as mildly elevated aminotransferases. Sonographic evaluation of the liver did not oxyclozanide reveal any abnormalities. Due to ongoing suspicion that the patient had PBC, a liver biopsy was performed that was nondiagnostic for PBC; however, immunostain for K19 highlighted focal bile duct loss and widespread loss of CoH (Table 1). The patient was subsequently started on 15 mg/kg daily dose of ursodeoxycholic acid (UDCA). During the follow-up period of 4 years, the patient’s AP, GGT, and aminotransferase levels normalized. The patient also responded symptomatically and reported resolution of complaints of pruritus and fatigue following initiation of treatment. There were no follow-up liver biopsies performed. Currently, the patient is still being treated and continues to be asymptomatic, with normal laboratory findings. Patient 2 also initially complained of pruritus.