Key Word(s): 1. acute pancreatitis; 2. outcomes; Presenting Author: LI PENG Corresponding Author: LI PENG Affiliations: The First Affiliated Hospital of Harbin Medical University Objective: To investigate the treatment of acute biliary pancreatitis (ABP). Methods: From January 2006 to December 2010, a total of 132 patients with ABP were admitted in the First Affiliated Hospital of Harbin Medical University Results: Among 132 cases, 128 patients were cured and 4 patients died (2 died in early stage, 2 died in later stage). The overall incidence of complication, Osimertinib price overall mortality and overall curative rate were 11.4% (15/13),

3.0% (4/132), and 97.0% (128/132), respectively. According to original disease we classified 132 patients with acute biliar pancreatitis: 9 patients (6.8%) with merely cholecystolithiasis, 61 patients (46.2%) with cholecystolithiasis combine common duct stones, 22 patients (16.7%) with merely common duct stones, 15 patients (11.4%) with cholecystolithiasis

combined choledochectasia, 19 patients (14.4%) with cholecystectomy combined common duct stones, other 6 patients (4.5%)(for instance: operated ERCP, calculus of intrahepatic Selleckchem Midostaurin duct et al). we evaluated every patients with APACHE II score in 48 h after admission, if the score <8 then patients were defined as patients with mild pancreatitis, if the score ≥8 points then the patients were defined as patients with severe type. According to obstructed or non-obstructed biliary tract and severity in pancreas, we divided patients into 4 groups: (1) 45 patients (34.1%) with mild non-obstruent type; (2) 53 patients (40.2%) with mild obstruent type; (3) 11 patients (8.3%) with severe non-obstruent type; (4) 23 patients (17.4%) with severe obstruent type. Conclusion: To improve the outcome of patients with acute

biliary pancreatitis, the individualized treatment based on the severity of pancreatitis should be considered. The two important factors that affect the outcome of this disease are indications and timing of intervention. learn more It is suggested that dealing with the pathology of biliary tract as soon as possible in order to prevent the recurrence of pancreatitis. Key Word(s): 1. Acute pancreatitis; 2. diagnosis; 3. treatment; Presenting Author: KEN ITO Corresponding Author: KEN ITO Affiliations: Toho University, Omori Medical Center Objective: The efficacy of electrohydraulic lithotripsy (EHL) is well documented for the treatment of chronic pancreatitis lithiasis when endoscopic lithotripsy failed. As an alternative method, we attempt extracorporeal shock wave lithotripsy (ESWL) on an outpatient basis in our institution. We retrospectively evaluated the efficacy of the EHL as a second attempt and that of ESWL on an outpatient basis as a third attempt for treatment of pancreatic duct stones.

Key Word(s): 1. acute pancreatitis; 2. outcomes; Presenting Author: LI PENG Corresponding Author: LI PENG Affiliations: The First Affiliated Hospital of Harbin Medical University Objective: To investigate the treatment of acute biliary pancreatitis (ABP). Methods: From January 2006 to December 2010, a total of 132 patients with ABP were admitted in the First Affiliated Hospital of Harbin Medical University Results: Among 132 cases, 128 patients were cured and 4 patients died (2 died in early stage, 2 died in later stage). The overall incidence of complication, Ku 0059436 overall mortality and overall curative rate were 11.4% (15/13),

3.0% (4/132), and 97.0% (128/132), respectively. According to original disease we classified 132 patients with acute biliar pancreatitis: 9 patients (6.8%) with merely cholecystolithiasis, 61 patients (46.2%) with cholecystolithiasis combine common duct stones, 22 patients (16.7%) with merely common duct stones, 15 patients (11.4%) with cholecystolithiasis

combined choledochectasia, 19 patients (14.4%) with cholecystectomy combined common duct stones, other 6 patients (4.5%)(for instance: operated ERCP, calculus of intrahepatic LY2606368 cell line duct et al). we evaluated every patients with APACHE II score in 48 h after admission, if the score <8 then patients were defined as patients with mild pancreatitis, if the score ≥8 points then the patients were defined as patients with severe type. According to obstructed or non-obstructed biliary tract and severity in pancreas, we divided patients into 4 groups: (1) 45 patients (34.1%) with mild non-obstruent type; (2) 53 patients (40.2%) with mild obstruent type; (3) 11 patients (8.3%) with severe non-obstruent type; (4) 23 patients (17.4%) with severe obstruent type. Conclusion: To improve the outcome of patients with acute

biliary pancreatitis, the individualized treatment based on the severity of pancreatitis should be considered. The two important factors that affect the outcome of this disease are indications and timing of intervention. for It is suggested that dealing with the pathology of biliary tract as soon as possible in order to prevent the recurrence of pancreatitis. Key Word(s): 1. Acute pancreatitis; 2. diagnosis; 3. treatment; Presenting Author: KEN ITO Corresponding Author: KEN ITO Affiliations: Toho University, Omori Medical Center Objective: The efficacy of electrohydraulic lithotripsy (EHL) is well documented for the treatment of chronic pancreatitis lithiasis when endoscopic lithotripsy failed. As an alternative method, we attempt extracorporeal shock wave lithotripsy (ESWL) on an outpatient basis in our institution. We retrospectively evaluated the efficacy of the EHL as a second attempt and that of ESWL on an outpatient basis as a third attempt for treatment of pancreatic duct stones.

The enzyme is then purified for administration. The results show that the biopharmaceutical is safe and many of the parameters of CESD, e.g., elevated transaminases, ferritins, and serum cholesterol levels, respond rapidly, and upon withdrawal of LAL they partially return to pretreatment levels. Although liver biopsies were not a part of this study, several patients had evidence of cholesterol mobilization with transient increases in serum cholesterol

following LAL administration. This result varied between patients and may be related to the degree of liver check details involvement, e.g., fibrosis. The exact mechanism of this effect is unknown, but likely relates to the delivery of LAL to lysosomes in hepatocytes and Kupffer cells since in rodents this is where most of the enzyme localizes. In future studies, the relationships between the serum cholesterol levels and hepatic histology could be important, as the peak of the cholesterol elevation following LAL administration might serve as a biomarker of excess cholesteryl esters

in the hepatocytes and other cell types. Similarly, the extent of reversibility of tissue lesions and residual damage will need to be assessed. Although not part of this safety study, a role click here of LAL therapy in WD appears clear since, except for one case, the effects of HSCT have been uniformly poor. However, the lethality of WD will make early and rapid diagnosis essential. The degree of recovery in WD, or CESD, is not known, but based on other rapidly progressive lysosomal storage diseases, e.g., Infantile Pompe disease, earlier therapy leads to better outcomes. As with the other lysosomal storage diseases for which enzyme replacement therapies are available, there remain many questions and issues to address as this therapy for

LAL deficiency states moves forward: how soon to treat, how to predict the disease severity, how much is reversible, what are the long-term effects, are all tissues treated equally, and the list goes on. Importantly, Nintedanib mw there are and will be many basic and applied issues that arise from this promising treatment. As with the other lysosomal storage diseases, LAL therapy should stimulate much basic and clinical research into these neglected diseases that will lead to enhanced overall care and improved health of afflicted patients. Gregory Grabowski, M.D. “
“Sexually transmitted infections continue to be a significant medical problem in men who have sex with men. Some of these patients will be seen in gastroenterology and hepatology clinics while others will attend clinics for sexually transmitted disorders. Although the recognition of acquired immunodeficiency syndrome (AIDS) was followed by the use of safer sex practices in the 1980s, epidemiologic studies now indicate increasing rates for at least some infections such as anorectal gonorrhea and syphilis. The common anorectal infections in homosexual men are herpes simplex virus, Neisseria gonorrhoeae, syphilis and Chlamydia trachomatis.

5% and 89.7%; P=NS), it was significantly greater in the TDF group for HBeAg-positive patients (78.8% and 50.6%, respectively; P<0.05). Over 48 weeks of treatment, virologic breakthrough occurred in only one patient in each group, and was not associated with emergence Selleckchem GSK1120212 of drug resistance. Univariate and subsequent multivariate logistic regression analyses indicated that TDF was an independent predictor of undetectable HBV DNA at 48 weeks (odds ratio [OR], 3.35; P<0.05), along with cirrhosis (1.61), baseline HBV DNA (0.52), HBeAg positivity (0.22),

age (0.98) and male gender (1.39; Ps<0.05). Conclusions Although TDF and ETV as initial antivirals have similar potency in viral suppression regardless of HBeAg positivity, TDF is superior in achieving HBV DNA negativity after 48 weeks of therapy in HBeAg-positive CHB patients. Longer follow-up data are needed to clarify whether the rapidity of viral suppression by these potent drugs affects ultimate viral clearance. Disclosures: Young-Suk Lim - Advisory Committees or Review Panels: Bayer Healthcare, Gilead Sciences; Grant/Research Support: Bayer Healthcare, BMS, Gilead Sciences, Novartis Han Chu Lee - Grant/Research Support: Medigen Biotechnology Co., Novartis, Roche, Bayer HealthCare, Bristol-Myers Squibb, INC research, Boehringer Ingelheim, Taiho Pharmaceutical

Co., Yuhan Co. The following people have nothing to disclose: Young Joo Yang, Ju Hyun Shim, Hyung-Don Kim, Yeonjung Ha, Mi-Jung Jun, Seung Bum Lee, Jee Eun Yang, Gi-Ae Kim, Eui Ju Park, Jihyun An, Danbi Lee, Kang Mo Kim, Young-Hwa Chung, Selleck AZD9291 Yung Sang Lee,

Dong Jin Suh BACKROUND/AIM: We evaluated the efficacy of tenofovir learn more (TDF)containing treatment and compared the outcome of the TDF monotherapy and TDF with nucleoside analogue (NA) in patients with suboptimal response (SOR) to ADF with or without NA in lamivudine (LMV) resistant CHB. METHODs: All study subjectshave received ADF with or without NAfor more than 6 months due to prior lamivudine resistance and then switched to TDF with or without NA due to SOR (HBV DNA >20 IU/ml after at least 6 months therapy). RESULT: A total of 125 patients were eligible. Previous therapy consisted of either ADF monotherapy(n=18) orADF with NA(LMV, telbivudine, clevudine and entecavir[ETV])(n=107).Overall cumulative proportion of CVR was 100 of 125 (80%) patients at 60 weeks of treatment. Nineof 13 patients (69.2%) with ADFresistance achieved CVR during 48 weeks and ADF mutation didn’t influence to CVR rate (P=0.732). During the follow up period of 48 weeks, Kaplan-Meier analysis showed no significant difference in CVR rate (P=0.706) andrepeated measured ANOVA analysis revealed no difference in the change of viral load (P=0.971)between TDF monotherapyand TDF with NA group. When we compare the patients with partial virologic response (PVR) and with non-response (NR), CVR rate was higher in patients with PVR at 12 weeks (P=0.

Although the peptide was detectable in the chimeric livers of these mice, no selective enrichment in the transplanted PHH was observed.21 To investigate whether HBVpreS/2-48myr is specifically directed to the liver we performed in vivo distribution studies of HBVpreS/2-48myr-y-125I in NRMI mice (Fig. 2A). To control specificity we included a randomized preS1-peptide (HBVpreS/2-48_scrstea-y-125I) (Fig. 2B).20 As depicted in Fig. 2A, 5 minutes after intravenous application of ∼45 μg HBVpreS/2-48myr-y-125I,

an almost exclusive accumulation of radioactivity in the liver was observed (>83%). The signal ceases slowly and was still detectable with ∼34% of the injected dose 24 hours postinjection (p.i.). The kinetic analysis revealed a half-life time of ∼18 hours. Apart from the pronounced liver accumulation we noticed a minor signal within the first 4 hours in the bladder. The signal was lost between 4 GSI-IX in vivo hours and 6 hours p.i., probably through renal clearance between repeated anesthesia. The percentage of radioactivity in the bladder after 4 hours was 16%. In contrast to the hepatic enrichment of HBVpreS/2-48myr-y-125I, intravenous

injection of the randomized HBVpreS/2-48scrstea-y-125I resulted in disperse body distribution. During the first anesthesia (0-4 hours) a higher percentage of the mutant peptide appeared in the bladder (30%). Six hours p.i. most of the radioactivity was eliminated; at 24 A-769662 order hours p.i. no peptide was detectable. Accordingly, the radioactivity was renally filtered with a half-life of about 4 hours. This was confirmed by the autoradiographic analysis of the corresponding liver sections (Fig. 2C). While an equal distribution of radioactivity in liver lobules was detectable 24 hours p.i. after application of the GNE-0877 wildtype peptide, no signal appeared for the mutant. This implies that the HBVpreS-lipopeptide accumulation in the

liver requires the integrity of the HBVpreS1-sequence, indicating a sequence-specific binding of the peptide to an HBVpreS-receptor. To map the sequence required for the accumulation of HBVpreS/2-48myr in the mouse liver we performed in vivo distribution analyses using the iodinated peptides depicted in Fig. 3D. These peptides have been characterized with respect to their ability to inhibit HBV infection.20 The results are summarized in the right column of Fig. 3D. To obtain quantitative measures for their ability to accumulate in different organs we injected the labeled peptides intravenously and sacrificed three mice per point in time (10 minutes, 1 hour, 4 hours, and 24 hours), extracted the intestine, brain, muscle, kidney, liver, spleen, lung, heart, and blood, counted the organ-associated radioactivity, and calculated the percentage of the injected dose per gram tissue (%ID/g). As shown in Fig. 3A, a modified (Q46K), genotype C-derived preS1-lipopeptide (Myrcludex B) locates with 71.

Attending to such cues, and using them to assess the physical attributes and condition both of potential competitors and mates, can have important

implications for the reproductive opportunities and survival of receivers. In addition to static attributes, cues to transient qualities such as emotional or motivation state and dynamic qualities such as reproductive status or dominance rank can also be advertised in the source and filter components of vocal signals. Moreover, there is growing evidence that in some primate species callers are able to produce vocalizations containing information about events or objects this website in the external world encoded in their source and filter-related component characteristics. Finally, we have discussed how the inter-individual variation in anatomy/physiology reflected in the acoustic

structure of vocal signals can lead to voice differences between individuals, and more specifically, how identity information can be given by frequency or amplitude contours, as is observed in the identifying whistles, and more generally in the ‘vocal signatures’, of several species. In conclusion, this review has highlighted the important Wnt antagonist contributions of the source–filter paradigm to understanding mammal vocal communication. Understanding call production mechanisms has enabled the development of a testable framework for the investigation of the origin and function of signals. This conclusion is illustrated in Fig. 5, which provides an overview of the evolutionary feedback loop linking production mechanisms to the acoustic structure of signals and the ultimate effect this has on the perception by, and behaviour

of receivers. Many thanks to Karen McComb and Ben Charlton for their helpful comments on earlier versions of the paper, and to Alan McElligott for his support throughout the writing process. Thanks also to the contributions of Tim Halliday and one anonymous referee. Funded by a BBSRC studentship to the first author. “
“We comprehensively reviewed information on maximum new life spans of wild birds (based on banding recoveries) and nine ecological, physiological and behavioral variables that have been hypothesized to affect the evolution of avian life spans. Data on maximum longevities and body masses were available for 936 species, and data on all variables were available for 470 species in 40 families from 15 orders. The Phoenicopteriformes (flamingos), Psittaciformes (parrots) and Procellariiformes (petrels and shearwaters) had the longest mean maximum life spans (>30 years), and the Passeriformes (perching birds), Podicipediformes (grebes) and Piciformes (woodpeckers) had the shortest mean maximum life spans (<10 years).

Attending to such cues, and using them to assess the physical attributes and condition both of potential competitors and mates, can have important

implications for the reproductive opportunities and survival of receivers. In addition to static attributes, cues to transient qualities such as emotional or motivation state and dynamic qualities such as reproductive status or dominance rank can also be advertised in the source and filter components of vocal signals. Moreover, there is growing evidence that in some primate species callers are able to produce vocalizations containing information about events or objects KU-57788 price in the external world encoded in their source and filter-related component characteristics. Finally, we have discussed how the inter-individual variation in anatomy/physiology reflected in the acoustic

structure of vocal signals can lead to voice differences between individuals, and more specifically, how identity information can be given by frequency or amplitude contours, as is observed in the identifying whistles, and more generally in the ‘vocal signatures’, of several species. In conclusion, this review has highlighted the important PI3K inhibitor contributions of the source–filter paradigm to understanding mammal vocal communication. Understanding call production mechanisms has enabled the development of a testable framework for the investigation of the origin and function of signals. This conclusion is illustrated in Fig. 5, which provides an overview of the evolutionary feedback loop linking production mechanisms to the acoustic structure of signals and the ultimate effect this has on the perception by, and behaviour

of receivers. Many thanks to Karen McComb and Ben Charlton for their helpful comments on earlier versions of the paper, and to Alan McElligott for his support throughout the writing process. Thanks also to the contributions of Tim Halliday and one anonymous referee. Funded by a BBSRC studentship to the first author. “
“We comprehensively reviewed information on maximum Racecadotril life spans of wild birds (based on banding recoveries) and nine ecological, physiological and behavioral variables that have been hypothesized to affect the evolution of avian life spans. Data on maximum longevities and body masses were available for 936 species, and data on all variables were available for 470 species in 40 families from 15 orders. The Phoenicopteriformes (flamingos), Psittaciformes (parrots) and Procellariiformes (petrels and shearwaters) had the longest mean maximum life spans (>30 years), and the Passeriformes (perching birds), Podicipediformes (grebes) and Piciformes (woodpeckers) had the shortest mean maximum life spans (<10 years).

Methods: FOXM1 expression in 52 clinical HCC tissues was examined both by immunohistochemistry and Western blot; Chromatin Immunoprecipitation (ChIP) was performed to examine the possible occupancy of the AFP promoter by FOXM1; Cytotoxicity of Thiostrepton were tested in HepG2 and HepG2.2.15 cell lines; cell cycle profiles were assessed by flow cytometry and the possible molecular targets were explored. Results: Up-regulation of FOXM1 was confirmed in 69.2% (36/52) of HCC tissues. Clinicopathologically, FOXM1 up-regulation was associated with metastasis (P=0.039). More importantly, a significant positive

correlation was observed PD-0332991 manufacturer between either tissue AFP mRNA or plasma AFP concentration and tissue FOXM1 expression levels BTK inhibitor in vitro before surgery (r=0.3, p=0.03; r=0.332, p=0.016; respectively). Meanwhile, a significant positive correlation between tissue AFP mRNA and pre-operative serum AFP level was demonstrated by the spearman rank correlation test (r=0.574, P<0.01). Both FoxM1 interference and Thiostrepton treatment led to significant reduction of AFP secretion in cell culture media. Moreover, CHIP assay confirmed that FOXM1 can bind to eight Forkhead response elements (FHREs) located at the proximal region of the AFP promoter. In addition, Thiostrepton dramatically reduced

FOXM1 expression in HCC cells, leading to cell cycle blockade at G1/S transition, concomitant with down-regulated CyclinD1, CDK2, CDK4 and SKP2 expression, and significantly induced FOXO3A expression. Conclusions: These data suggested that FOXM1 expression is closely correlated with both in vitro and in vivo AFP production possibly through its transcriptional regulation of the AFP promoter. Thiostrepton may specifically target FOXM1 to induce cytotoxic effect and

could be potentially developed as a novel anticancer drug against HCC. Disclosures: The following people have nothing to disclose: Huang S. Feng, Ai J. Gang, Wu Yan, Chen J. Juan, Liping Zhang Background and Aims: Liver is the most common site of metastasis for pancreatic cancer. In tumor microenvironment, MG-132 datasheet stellate cells influence growth and metastasis of cancer by multiple mechanisms, including regulating extracellular matrix turnover. Sphingosine 1-phosphate (S1P) signaling has been implicated in tumorigenesis and metastasis in many cancers; however, its role in the metastasis of pancreatic cancer cells to the liver, remains unexplored. We hypothesized that S1P activates stellate cells to release paracrine factors that promote tumor cell migration and invasion that promote metastatic growth. Methods: Immortalized human or mouse stellate cells were stimulated with S1P (0.5–5 μM) or vehicle. S1 P1 or S1P2 receptor was disrupted by shRNA, siRNA, or pharmacological inhibitors. Stellate cell conditioned media was used to measure pancreatic cancer cell (PANC1) migration and invasion in Boy-den chamber and Transwell assays.

Methods: FOXM1 expression in 52 clinical HCC tissues was examined both by immunohistochemistry and Western blot; Chromatin Immunoprecipitation (ChIP) was performed to examine the possible occupancy of the AFP promoter by FOXM1; Cytotoxicity of Thiostrepton were tested in HepG2 and HepG2.2.15 cell lines; cell cycle profiles were assessed by flow cytometry and the possible molecular targets were explored. Results: Up-regulation of FOXM1 was confirmed in 69.2% (36/52) of HCC tissues. Clinicopathologically, FOXM1 up-regulation was associated with metastasis (P=0.039). More importantly, a significant positive

correlation was observed RAD001 order between either tissue AFP mRNA or plasma AFP concentration and tissue FOXM1 expression levels Serine Protease inhibitor before surgery (r=0.3, p=0.03; r=0.332, p=0.016; respectively). Meanwhile, a significant positive correlation between tissue AFP mRNA and pre-operative serum AFP level was demonstrated by the spearman rank correlation test (r=0.574, P<0.01). Both FoxM1 interference and Thiostrepton treatment led to significant reduction of AFP secretion in cell culture media. Moreover, CHIP assay confirmed that FOXM1 can bind to eight Forkhead response elements (FHREs) located at the proximal region of the AFP promoter. In addition, Thiostrepton dramatically reduced

FOXM1 expression in HCC cells, leading to cell cycle blockade at G1/S transition, concomitant with down-regulated CyclinD1, CDK2, CDK4 and SKP2 expression, and significantly induced FOXO3A expression. Conclusions: These data suggested that FOXM1 expression is closely correlated with both in vitro and in vivo AFP production possibly through its transcriptional regulation of the AFP promoter. Thiostrepton may specifically target FOXM1 to induce cytotoxic effect and

could be potentially developed as a novel anticancer drug against HCC. Disclosures: The following people have nothing to disclose: Huang S. Feng, Ai J. Gang, Wu Yan, Chen J. Juan, Liping Zhang Background and Aims: Liver is the most common site of metastasis for pancreatic cancer. In tumor microenvironment, 3-mercaptopyruvate sulfurtransferase stellate cells influence growth and metastasis of cancer by multiple mechanisms, including regulating extracellular matrix turnover. Sphingosine 1-phosphate (S1P) signaling has been implicated in tumorigenesis and metastasis in many cancers; however, its role in the metastasis of pancreatic cancer cells to the liver, remains unexplored. We hypothesized that S1P activates stellate cells to release paracrine factors that promote tumor cell migration and invasion that promote metastatic growth. Methods: Immortalized human or mouse stellate cells were stimulated with S1P (0.5–5 μM) or vehicle. S1 P1 or S1P2 receptor was disrupted by shRNA, siRNA, or pharmacological inhibitors. Stellate cell conditioned media was used to measure pancreatic cancer cell (PANC1) migration and invasion in Boy-den chamber and Transwell assays.

“
“Cystic fibrosis liver disease (CFLD), which results from progressive hepatobiliary fibrosis, MG-132 solubility dmso is an important cause of morbidity and mortality, but it is difficult to identify before portal hypertension (PHT) ensues. Clinical signs, serum alanine aminotransferase (ALT) levels, and ultrasound (US) are widely

applied, but their value in predicting the presence of cirrhosis, the development of PHT, or adverse outcomes is undetermined. The potential gold standard, liver biopsy, is not standard practice and, notwithstanding sampling error considerations, has not been systematically evaluated. Forty patients with cystic fibrosis (median age = 10.6 years) with abnormal clinical, biochemical, www.selleckchem.com/products/abc294640.html and US findings were subjected

to dual-pass percutaneous liver biopsy. Clinical outcomes were recorded over 12 years of follow-up (median = 9.5 years for survivors). Logistic regression and receiver operating characteristic analyses were applied to predict hepatic fibrosis (which was assessed by fibrosis staging and quantitative immunohistochemistry) and the occurrence of PHT. PHT occurred in 17 of 40 patients (42%), including 6 of 7 (17%) who died during follow-up. Clinical examination, serum ALT levels, and US findings failed to predict either the presence of liver fibrosis or the development of PHT. Fibrosis staging on liver biopsy, where the accuracy was improved by dual passes (P = 0.002, nonconcordance = 38%), predicted the development of PHT (P < 0.001), which occurred more frequently and at a younger age in those with severe fibrosis. Conclusion: Clinical modalities currently employed

to evaluate suspected CFLD help to identify a cohort of children at risk for liver disease and adverse outcomes but do not predict an individual’s risk of liver fibrosis or PHT development. Liver fibrosis on biopsy predicts the development of Cyclooxygenase (COX) clinically significant liver disease. Dual passes help to address sampling concerns. Liver biopsy has a relevant role in the management of patients with suspected CFLD and deserves more widespread application. (HEPATOLOGY 2011) Hepatobiliary fibrosis causes significant mortality and morbidity in patients with cystic fibrosis (CF).1 Liver cirrhosis has been reported in up to 10% of children2 and in less than 2% of adults with CF3; suggesting a survival disadvantage. Liver disease is reportedly the third leading direct cause of death.3 Complications such as portal hypertension (PHT), nutritional growth failure, and, in some cases, liver synthetic failure impair the quality of life in up to 20% of patients1, 4; this is also highlighted by improvements in the quality of life, nutritional status, and respiratory function after successful liver transplantation.1, 4 Cystic fibrosis liver disease (CFLD) has its origins early in life,1, 4 and its onset and progression to cirrhosis and PHT are unpredictable.