Signs Of Cirrhosis Research Articles

Liver Transplantation for Hepar Lobatum with Clinical Manifestations of Cryptogenic Cirrhosis

Introduction: Hepar Lobatum (HL) is a chronic liver disorder mimicking cirrhosis, associated with liver metastases, the breast being the most common primary site. Non-malignant causes can also result in HL, tertiary syphilis and schistosomiasis described the most. The pathogenesis of this condition is related to occlusions of intrahepatic portal branches by neoplastic thrombus or desmoplastic changes, especially in patients undergoing tumor regression by chemotherapy. May be associated with portal hypertension. The shrinkage of the hepatic parenchyma involving one or more lobes occurs in about 13% of cirrhotic livers, 50% of the livers courses with portal hypertension. Results: Female, 42 years old, diagnosed with cryptogenic cirrhosis started after an episode of frank hematemesis and melena in November 2010. Presented with ascites and encephalopathy grade I during follow up. She underwent orthotopic liver transplantation on May 16, 2011, MELD 24, by Piggy Back technique. During the surgery there was moderate ascites, splenomegaly, other signs of portal hypertension, multi lobulated liver, size reduced, with structural changes consistent with hepar lobatum. Patient responded well to immediate postoperative. Because of the intraoperative finding compatible with hepar lobatum, syphilis and other infectious diseases serology were requested, being all negative. The total hepatectomy product weighed 630g, presenting heavily anatomical deformities by the capsular surface, with presence of multiple nodular areas and deep grooves, sometimes pedunculated or totally detached from the body and wide variation in diameter. In microscopic examination, it was found intense variability of histological changes in liver parenchyma, no signs of cirrhosis and portal spaces expanded by fibrosis, with the issuance of septa and sketches of nodules. Intensely anomalous hyperplastic portal vessels were also commonly observed. Conclusion: Currently, it is known that the hepar lobatum is associated with liver involvement in tertiary syphilis, Hodgkin's lymphoma and in cases of metastatic breast cancer, probably due to vascular obstruction with subsequent loss of liver volume. Cases post- chemotherapy for metastatic carcinoma are also described, with fibrosis and retraction of the parenchyma. In this case, we observed morphological changes unrelated to infectious diseases or neoplasia. Due to the observation of peculiar form of distribution of portal vessels in this case, we question whether the same could not be among the primary events causing the hepar lobatum.

E-Manuscript Article Summaries *

Pseudocirrhosis is a rare form of liver disease that causes clinical symptoms and shows radiographic signs of cirrhosis, but that has histologic features suggesting a distinct pathologic process[...].

The Correlation of Il28B Genotype With Sustained Virologic Response In Romanian patients With Chronic Hepatitis C

Multiple variables influencing the sustained virologic response (SVR) in chronic hepatitis C have been evaluated. One of them is genetic polymorphism near the IL28B gene. The aim of this study was to evaluate the influence of IL28B genotypes on SVR rates in a group of patients with chronic hepatitis C from the western part of Romania. A retrospective study was performed in 107 consecutive patients, previously treated with standard-of-care medication for chronic hepatitis C, identified from the databases of 2 centers. Patient demographics, viral load before treatment and at 12, 24, and 72 weeks from the treatment start, and IL28B genotype were evaluated. Among the 107 patents in the study group, 54 patients had SVR (50.5%), and 62 (57.9%) showed a complete early virologic response (cEVR). The SVR rates according to IL28B genotype were as follows: 73.1% in patients with genotype C/C, 40.9% in those with genotype C/T, and 57.1% in those with genotype T/T (i.e., 73.1% among patients with the C/C genotype vs. 43.7% among those with non-C/C genotypes; P = 0.0126). The cEVR rates were 80.8% in patients with the C/C genotype vs. 51.2% in those with non-C/C genotypes (P = 0.011). In our cohort of 107 Caucasian HCV patients, the SVR rate was 50.5% with standard-of-care treatment. The SVR rate was directly related to the IL28B genotype: 73.1% in the C/C genotype vs. 43.7% in non-C/C genotypes (P = 0.0126).

Amniotic Membrane Application Reduces Liver Fibrosis in a Bile Duct Ligation Rat Model

Biliary fibrosis and resultant cirrhosis are among the most common outcomes of chronic liver diseases. Currently, liver transplantation remains the only effective treatment. In seeking alternative therapeutic approaches, we focused on the potential use of the human amniotic membrane (AM). Indeed, AM has gained increasing importance for its antiscarring, anti-inflammatory, and wound-healing properties, as well as for the multipotent differentiation ability and immunomodulatory features of AM-derived cells. Intriguingly, we have recently demonstrated that placenta-derived cells reduce lung fibrosis in bleomycin-treated mice, and that AM patches reduce postischemic cardiac injury in rats. Hence, we have now investigated the effects of human AM on biliary fibrosis induced in rats through the bile duct ligation (BDL) procedure. A fragment of human AM was applied onto the liver surface after BDL and the effects on fibrosis establishment and progression were evaluated at different time points in comparison with fibrosis progression in control BDL rats. The degree of liver fibrosis was first assessed by the semiquantitative Knodell scoring system and, thereafter, by digital image morphometric analysis to quantify the area occupied by ductular reaction, activated myofibroblasts, and collagen deposition. We demonstrated a significant reduction in the severity of BDL-induced fibrosis in AM-treated rats. Indeed, while fibrosis progressed rapidly in control BDL rats, leading to cirrhosis within 6 weeks, AM-treated rats showed confined fibrosis at the portal/periportal area with no signs of cirrhosis, and a reduction in collagen deposition to about 50% of levels observed in control BDL rats. In addition, the AM was able to significantly slow the gradual progression of the ductular reaction and reduce, at all time points, the area occupied by activated myofibroblasts. These findings suggest that human AM, when applied as a patch onto the liver surface, might inhibit fibrosis progression in BDL-injured livers, and could protect against hepatic damage associated with fibrotic degeneration.

345 A NEW METHODOLOGY FOR ASSESSING PROGRESSION OF LIVER FIBROSIS IN LARGE POPULATIONS USING VALIDATED BIOMARKER FIBROTEST® INSTEAD OF BIOPSY

Methods: The study group included 471 patients (p): 83 subjects without liver fibrosis (82 healthy volunteers, considered F0 Metavir and 1 patient with F0 in liver biopsy-LB), 13 p with F1 in LB, 25p with F2, 28p with F3 and 322p with liver cirrhosis (15p with LB and 307p with clinical, ultrasonographic, endoscopic and/or laparoscopic signs of cirrhosis). We performed 10 measurements in each patient expressed in meters/second (m/s) and a median value was obtained. We considered good technical parameters IQR< 30% and SR≥60%, similar to Transient Elastography. For statistical analysis we used the Spearman rank correlation coefficient (r). Results: In 11p we did not obtained valid ARFI measurements (2.3%) and in 45p (9.7%) of 460p with valid measurements, the technical parameters were unsatisfactory. In the study group, there was a direct, strong correlation (r = 0.744) between ARFI values and liver fibrosis (p < 0.0001); depending on technical parameters the correlation was: for IQR < 30% and SR≥60%: r = 0.779, p < 0.0001 and for IQR≥30% and/or SR< 60%: r = 0.268, p = 0.07. Conclusion: The SR and IQR influence the values of LS measured by ARFI, thus, as in Transient Elastography, we should propose reliable only the measurements with IQR < 30% and SR≥60%.

Giant Cell Hepatitis: an Unusual Cause of Fulminant Liver Failure

Giant cell hepatitis is a very rare disease of unknown origin. It has been hypothesized that drugs, viral infections, or autoimmune reactions may play a pathogenetic role. Here, we describe a 33 year old patient with bacterial bronchitis who was treated with doxycycline (100 mg/d) for one week. Furthermore the patient complained of malaise and a distinct jaundice. Liver parameters increased dramatically (AST 4670 U/l, ALT 5350 U/l, bilirubin 226 µmol/l) and liver function was impaired (INR = 1,45). The ultrasound scan showed a hepatomegaly with no signs of cirrhosis, normal spleen size and normal bile ducts; liver perfusion was normal. No evidence of Wilson's disease, hemochromatosis, autoimmune hepatitis, primary sclerosing cholangitis, primary biliary cirrhosis, hepatitis A, B, C and E, HIV, CMV, VZV, adenoviral infections, or paracetamol intoxication was found. Subsequently, the patient developed acute liver failure (AST 2134 U/l, ALT 2820 U/l, bilirubin 380 µmol/l, INR 3.0) and a beginning renal failure. Therefore, he was transferred to our transplant center. Due to increasing confusion and somnolence due to cerebral edema mechanical ventilation was needed. Because of an acute renal failure and severe hepatic encephalopathia MARS-hemodialysis was performed. Three weeks after the appearance of the jaundice he underwent liver transplantation (MELD 40). Surprisingly, in the explanted liver the diagnosis of giant cell hepatitis was made. Today--2 years after successful liver transplantation--the patient is in very good condition with normal liver function. In conclusion, giant cell hepatitis is a rare cause of acute liver failure that is often recognized only histologically.

Schwere Hepatopathie und subakutes Leberversagen mit „Fast-track”-Leberzirrhose bei älterer Patientin

We report the case of a 74-year-old lady who presented at our clinic with icterus and cholestatic hepatitis. For atrial fibrillation she had been prescribed a medication with phenprocoumone. After ruling out viral, autoimmune, and metabolic causes of hepatitis, we performed a liver biopsy which led to the diagnosis of phenprocoumone-related liver damage. The patient was discharged without phenprocoumone and completely compensated liver function. Five weeks later she returned to the hospital with encephalopathy, ascites, coagulopathy, varices, and signs of cirrhosis in abdominal ultrasound. In spite of treatment with steroids, the patient died of subacute liver failure several weeks later. This case illustrates the occasionally poor course of toxic hepatitis even after discontinuation of the responsible medication, potential treatment options are discussed.

Hepatic Vein Morphology

The purpose of this study was to evaluate the accuracy of a new sonographic marker for the diagnosis of cirrhosis using hepatic vein wall changes. A prospective pilot study evaluating 88 patients, 38 with cirrhosis and 50 with no evidence of liver disease, was undertaken. Hard copy sonograms of the hepatic veins were obtained and reviewed in a blinded fashion by 2 radiologists. The hepatic vein morphology was assessed by 3 parameters: hepatic vein wall straightness, uniformity of hepatic vein wall echogenicity, and visualization of a complete 1-cm hepatic vein segment. The 3 parameters were compared to evaluate sensitivity and specificity for the diagnosis of cirrhosis. Interobserver and intraobserver errors for each parameter were also calculated with kappa statistics to assess reproducibility. There was a strong correlation between altered straightness and nonuniformity of hepatic vein wall echogenicity and cirrhosis. The straightness parameter had superior sensitivity of 97% (95% confidence interval [CI], 85%-100%) and specificity of 91% (95% CI, 78%-97%) for diagnosis of cirrhosis. Uniformity of hepatic vein wall echogenicity was the next most useful parameter, with sensitivity of 88% (95% CI, 73%-97%) and specificity of 86% (95% CI, 72%-95%). The continuous 1-cm segment of the hepatic vein had sensitivity of 68% (95% CI, 49%-83%) and specificity of 91% (95% CI, 78%-97%). Hepatic vein evaluation was found to show both good intraobserver and interobserver error. Hepatic vein morphology on sonography, in particular, changes in the straightness and uniformity of hepatic vein wall echogenicity, is a new sign of cirrhosis, which may increase the overall accuracy of sonographic diagnosis of cirrhosis and which appears to have a moderately high degree of reproducibility.

Clinical Challenges and Images in GI

Question: A 29-year-old woman with no previous medical history presented with abdominal pain and a palpable mass in the upper abdomen. Physical examination confirmed the presence of a hard mass in the epigastrium. Biochemical and hematologic tests (blood urea nitrogen, electrolytes, liver function panel, C-reactive protein, and complete blood count) were within normal laboratory range. Abdominal computed tomography showed a 5- × 7-cm tumor in the upper abdomen, with areas of calcification and cystic transformation (Figure A). The liver had no signs of cirrhosis. No other lesions or pathologic findings were observed. Laparotomy showed a tumor in the ligamentum teres hepatis (Figure B), with expansive growth, covered by a thin fibrotic capsule, and sparing the liver. It was removed intact. What is your diagnosis? Look on page 2416 for the answer and see the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. Histologic examination revealed strong positive staining for HMB 45 (Figure C), with weaker staining for Melanin A, CD 99, and smooth muscle markers (Aktin). Cytoceratin, CD34, and CD117 were negative. Proliferation index (KI-67) was <5%. A diagnosis of PEComa of the ligamentum teres hepatis was made. PEComas are very rare pathologic entities, predominantly affecting females.1Martignoni G. Pea M. Reghellin D. et al.PEComas: the past, the present and the future.Virchows Arch. 2008; 452: 119-132Crossref PubMed Scopus (372) Google Scholar They have been identified at multiple anatomic sites, such as the liver, uterus, vulva, rectum, heart, breast, common bile duct, urinary bladder, abdominal wall, and pancreas. To the best of our knowledge, there are only 9 reported cases of PEComas of the falciform ligament/ligamentum teres.1Martignoni G. Pea M. Reghellin D. et al.PEComas: the past, the present and the future.Virchows Arch. 2008; 452: 119-132Crossref PubMed Scopus (372) Google Scholar, 2Tanaka Y. Ijiri R. Kato K. et al.HMB-45/melan-A and smooth muscle actin-positive clear-cell epithelioid tumor arising in the ligamentum teres hepatis: additional example of clear cell ‘sugar’ tumors.Am J Surg Pathol. 2000; 24: 1295-1299Crossref PubMed Scopus (67) Google Scholar, 3Folpe A.L. Mentzel T. Lehr H.A. et al.Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature.Am J Surg Pathol. 2005; 29: 1558-1575Crossref PubMed Scopus (646) Google Scholar They were more common in female individuals <30 years of age, not always involved the liver; some were associated with tuberous sclerosis.1Martignoni G. Pea M. Reghellin D. et al.PEComas: the past, the present and the future.Virchows Arch. 2008; 452: 119-132Crossref PubMed Scopus (372) Google Scholar Preoperative differential diagnosis includes teratomas, leiomyosarcomas, clear cell sarcomas of tendons and aponeuroses, and gastrointestinal stromal tumors. The macroscopic features of PEComas are nonspecific, such as focal hemorrhagic and cystic areas. They usually have a thin capsule, and nearly all are positive for melanocytic (HMB-45 and melan-A) and smooth muscle (actin and/or desmin) markers.1Martignoni G. Pea M. Reghellin D. et al.PEComas: the past, the present and the future.Virchows Arch. 2008; 452: 119-132Crossref PubMed Scopus (372) Google Scholar, 3Folpe A.L. Mentzel T. Lehr H.A. et al.Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature.Am J Surg Pathol. 2005; 29: 1558-1575Crossref PubMed Scopus (646) Google Scholar Malignant PEComas contain areas of necrosis, usually show a high mitotic index, and can develop lymphogenic and hematogenic (lungs, liver, bone) metastases. From the 9 reported cases of PEComa of the falciform ligament/ligamentum teres, 1 patient developed presumed lung metastases but died from another reason at 1 year,1Martignoni G. Pea M. Reghellin D. et al.PEComas: the past, the present and the future.Virchows Arch. 2008; 452: 119-132Crossref PubMed Scopus (372) Google Scholar and excision of the recurrent disease was performed in the second case, which was reported alive at 35 months.3Folpe A.L. Mentzel T. Lehr H.A. et al.Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature.Am J Surg Pathol. 2005; 29: 1558-1575Crossref PubMed Scopus (646) Google Scholar The authors thank Dr Andreas Kreft and Prof. Thomas J. Musholt for their contribution to this work.

The ultrasound examination in patients with jaundice – the experience of a tertiary internal medicine center

Background: The rapid and accurate diagnosis of patients with abdominal pain and jaundice is always challenging in clinical practice. The aim of the study is to evaluate the role of the ultrasound examination performed in the emergency room in these patients. Material and methods: We included in this retrospective study 256 patients who presented between 2005–2007 in the emergency room with abdominal pain and jaundice. In all patients, clinical examination, the standard biochemical workup and the standard abdominal ultrasound were performed. Results: In the emergency room, the ultrasonography offered a clear diagnosis in 188 cases (73.4%) and the distribution of these cases was: acute cholecystitis: 46 cases (27 linked to the presence of the gallstones); gallbladder cancer: 1 patient; obstruction of commune bile duct by gallstones, intraductal tumors, pancreatic tumors, lymphnodes: 34 cases; portal vein thrombosis with signs of cirrhosis or presence of digestive tumors: 27 cases; liver tumors (primitive or metastases): 23 cases; liver abscesses (after liver trauma or associated with tumoral necrosis): 15 cases; acute pancreatitis: 42 cases. In 37 cases we found only indirect signs, which correlated with clinical and biological data suggested the diagnosis: signs of portal hypertension in cirrhosis or caval and suprahaepatic vein distension in congestive heart failure. Other imaging techniques were necessary for the diagnostic in 31 cases with ultrasound abnormalities but without an evident cause: cholangio-MRI (revealing stones or tumors) or endoscopic retrograde cholangiopancreatography (revealing stenosis of the Oddi's sphincter) in patients with dilated common bile duct and computed tomography in patients with inhomogeneous liver or pancreas (revealing tumors). Conclusions: Ultrasonography, as a noninvasive and feasible method, performed in the emergency room, has a diagnostic value in almost three quarters of patients with abdominal pain and jaundice. In these patients it should be the first choice imaging method but, in some cases, other techniques are required to complete the diagnosis.

A Forty-One Year Old Man Died of Hepatocellular Carcinoma from Hepatitis B

Hepatocellular carcinoma (HCC) is a primary liver malignancy, the global incidence is about 15 cases per incidence of HCC 137,000 new cases. 1 The most important risk factor is viral hepatitis, especially hepatitis B. 2-3 Among Chinese population, 15-20% is described as HBV carriers (hepatitis B surface antigen positive). The relative risk of HCC among Hepatitis B carriers is 223 times that of non-carriers. These chronic carriers are usually not symptomatic and have normal liver function. Despite this, they are at risk of developing Hepatocellular carcinoma without signs of cirrhosis, active or advanced hepatitis. Unfortunately, the diagnosis is usually late 4-5 and the prognosis is generally poor, as the case described above.

Liver stiffness measurement for diagnosis of portal hypertension-related digestive bleeding in the ICU

Without past history or clinical signs of cirrhosis, the diagnosis of portal hypertension (PHT)-related digestive haemorrhage (DH) is often delayed until upper intestinal endoscopy performance, thus delaying specific medical treatment such as vasoactive drugs. As the liver stiffness measurement (LSM) is correlated to hepatic fibrosis [1] and PHT, it could be therefore useful for the diagnosis of cirrhosis in this situation. The aim of this study was to assess the predictive value of LSM by FIBROSCAN® for the diagnosis of cirrhotic PHT-related DH.

Autologous bone marrow cells in the treatment of cirrhosis of the liver

The data characterizing tolerance and efficiency of autologous bone marrow cells in the treatment of patients with cirrhosis of the liver are presented. Injection of autologous bone marrow cells was not associated with the development of adverse reactions. Cell therapy of patients with compensated cirrhosis arrested asthenic syndrome, reduced cytolysis, increased the level of serum albumin and platelet count. Ultrasonic examination revealed reduction of portal hypertension (the area of the spleen and the portal vein lumen decreased). In patients with decompensated cirrhosis, a positive response presenting as reduction of the disease severity (by 1.9 points) was observed in 48.6% cases. Positive shifts in these patients were associated with a decrease of ALT and AST levels, reduction of laboratory signs of cirrhosis, increase in platelet count, and reduction of the asthenic syndrome. Hence, therapy with autologous bone marrow cells is safe and, according to preliminary results, can be regarded as a new approach to the treatment of patients with cirrhosis of the liver.

La stéatose hépatique : une maladie émergente chez les patients infectés par le VIH

Hepatic steatosis is an emerging cause of morbidity in antiretroviral therapy (ART)-experienced HIV patients. The influence of steatosis on fibrosis is poorly understood. We report two cases of rapid evolution of disseminated macrovacuolar steatofibrosis to cirrhosis. Both patients had no history of alcohol abuse, nor intravenous drug use and were tested negative for HCV (PCR RNA) and had no HBS antigen. Patient 1 had a past history of hypertrygliceridemia, but controlled with dietetic measures for 4 years prior to biopsy. The first hepatic biopsy showed a disseminated macrovacuolar steatosis (>80%). The patient had then cytolysis and an uncontrolled HIV viral load. The second biopsy was performed two years later, and HIV was controlled by a new line of ART. It showed a regression of the steatosis (10%) and a progression of the fibrosis with signs of cirrhosis. Patient 2 had a long history of HIV infection. He also had an uncontrolled dyslipidemia. The first biopsy was realised during a period of uncontrolled HIV infection and elevated liver enzymes. The biopsy showed a major macrovacuolar steatosis (>80%). The second biopsy was realised 6 years after and showed the same steatosis and signs of cirrhosis. The HIV infection was then under control. Observations reported here show a rapid evolution of liver steatosis to cirrhosis in HIV positive / HCV negative patients, despite the control of HIV infection. The implication of HAART remains unclear. In the era of HAART, liver steatosis can rapidly evoluate to cirrhosis without any risk factor except ART.

Liver Transplantation in a Patient With Sitosterolemia and Cirrhosis

Sitosterolemia (MIM 210250) is a rare genetic disorder caused by disruption of the normal mechanisms that regulate dietary cholesterol absorption and prevent the accumulation of noncholesterol sterols. As a result of this defect, affected individuals accumulate high concentrations of plant sterols in plasma and tissues. They present clinically with tendon or tuberoeruptive xanthomas, premature coronary artery disease, and/or hemolytic anemia. Two genes, ABCG5 and ABCG8, compose the STSL locus, and complete mutation in either, but not both, results in disease. The expression of these genes is confined to the intestine and liver. They are thought to function as sterol efflux pumps. It is not clear which organ, liver or intestine, is of greater importance in maintaining sterol balance with respect to noncholesterol sterols. We report here a case of a patient who presented with "chronic active liver disease" and was found to have sitosterolemia by chance and subsequently underwent orthotopic liver transplantation. Following transplantation, the grossly elevated pretransplantation serum plant sterol levels decreased to values only slightly higher than those of the patient's heterozygous father. This case highlights 2 important features: (1) The liver functions as the predominant organ for maintaining noncholesterol sterol balance (because the intestinal defect was not altered), and (2) a new clinical feature of undiagnosed sitosterolemia may be "idiopathic" liver disease. Because the diagnosis of sitosterolemia is based on specialized plasma analyses, we would propose that some consideration to this diagnosis should be given in appropriate cases.

Effects of Indomethacin on Viral Replication Markers in Asymptomatic Carriers of Hepatitis B: A Randomized, Placebo-Controlled Trial

Previous studies have suggested some benefits of nonsteroidal antiinflammatory drugs (NSAIDs) use in patients with chronic viral hepatitis. We evaluated potential effects of indomethacin in asymptomatic carriers of hepatitis B surface antigen (HBsAg). Randomized, placebo-controlled, double-masked clinical trial. One hundred and twelve patients who were confirmed to be HBsAg carriers for at least 6 months and had normal liver function tests, normal abdominal sonography, and no sign of cirrhosis were randomly assigned into two groups. One group (56 participants, mean age (+/-SD) 31.7 (+/-9.6) yr, 29 male, mean serum alanine aminotransferase (ALT) (+/-SD) 24.9 (+/-9.2)) received indomethacin capsules (25 mg) three times daily and the other group (56 participants, mean age (+/-SD) 33.8 (+/-10.2) yr, 33 male, mean serum ALT (+/-SD) 24.5 (+/-8.7)) took placebo capsules with identical package and appearance. All participants were under treatment for 6 months and were followed 3 months thereafter. Statistical analyses were performed both by intention-to-treat and on-treatment methods. Nine participants in the indomethacin group (16%) and 8 in the placebo group (14%) did not complete the trial. HBsAg seroconversion did not differ by treatment group (2 subjects in each group became seronegative). Hepatitis B virus DNA (HBV-DNA) became negative in sera of 7 participants in the indomethacin group but only in 1 in the placebo group (intention-to-treat p= 0.06; on-treatment p= 0.03). Seroconversion of HBeAg to anti-HBe occurred only in 5 participants in the indomethacin group (intention-to-treat p= 0.06; on-treatment p= 0.03). Adverse events included one case of hepatotoxicity and two cases of gastritis in the indomethacin group and one suspected gastritis in the placebo group. We suggest use of indomethacin only in the subgroup of asymptomatic HBsAg carriers who have detectable HBV-DNA or HBeAg in their sera.

Non Cirrhotic Portal Fibrosis

Diagnosis of non cirrhotic portal fibrosis was considered when the following criteria were fulfilled evidence of portal hypertension (oesophageal varices, hypersplenism, ascites, or increased hepatic venous pressure gradient), Doppler ultrasound showing patent portal and hepatic veins, and liver biopsy showing sign of cirrhosis. Non cirrhotic portal fibrosis clinically characterized by splenomegaly, anemia, portal hypertension, and histopathological examination portal tract showing fibrosis and sclerosis. Portal hypertension are most caused by a cirrhotic liver (85%), there are only a few reports on non cirrhotic portal hypertension, mostly in Japan and India. We reported a case of non cirrhotic portal fibrosis in young male. The clinical complications of portal hypertension are variceal bleeding and pancytopenia due to hypersplenism. Variceal band ligation and splenectomy were performed. The patient showed good clinical response. Keywords: portal hypertension, non cirrhotic portal fibrosis, young male

Features and outcome of autoimmune hepatitis type 2 presenting with isolated positivity for anti-liver cytosol antibody

Autoimmune hepatitis (AIH) type 2 is identified by the presence in the serum of anti-liver/kidney microsome type 1 autoantibody. Anti-liver cytosol autoantibody has been reported in children with autoimmune liver disorders mostly in association with anti-liver/kidney microsome reactivity. However, its role as a sole marker of AIH type 2 is debated. We describe here a series of 18 children and adolescents (15 girls, 3 boys) with AIH with serum anti-liver cytosol type 1 (aLC1) as the only autoimmune marker. A retrospective review was conducted from 3 pediatric hepatology units of all children with an autoimmune liver disease associated with aLC1 as found by immunofluorescence and/or immunodiffusion or immunoblotting. Age at first symptoms ranged from 11 months to 14 years; 12 children presented with acute hepatitis, 1 with progressive jaundice, and 5 were asymptomatic. Anti-liver/kidney microsome, antimitochondria, and anti-actin autoantibodies were not detected. Signs of cirrhosis were present in 11 children. Immunosuppressive treatment was effective in all except 2 children who had subfulminant hepatic failure and who required liver transplantation. Sixteen patients (14 with their native liver) currently are alive; 14 patients still are on immunosuppressive therapy after 1 to 22 years. According to the international scoring system for the diagnosis of AIH, 16 patients corresponded to a definite diagnosis and 2 corresponded to a probable diagnosis. The presence of aLC1 in children with acute or chronic liver disease of unknown origin strongly supports a diagnosis of AIH and is an indication for early immunosuppressive therapy.

Health-state utilities and quality of life in hepatitis C patients.

Health-state utilities are global measurements of quality of life on a scale from 0 (death) to 1 (full health). Utilities are used to evaluate health outcomes and are the preferred outcome measure for policy models that determine the cost-effectiveness of treatments. Currently, utilities for hepatitis C virus (HCV)-infected patients have been estimated using expert judgments. The purpose of this study was to elicit HCV utilities directly from patients. We assessed the utilities of 193 outpatients at various stages of chronic HCV progression by using a visual analog scale, the standard gamble technique, the Health Utilities Index Mark 3 survey, and the EuroQol Index survey. We also incorporated the nonutility-based Short Form-36v2 survey, which provides a detailed profile of health status. The mean standard gamble utilities were: 0.78 for patients without a recent liver biopsy and no signs of cirrhosis; 0.79 for mild to moderate chronic HCV infection; 0.80 for compensated cirrhosis; 0.60 for decompensated cirrhosis; 0.72 for hepatocellular carcinoma; 0.73 for transplant; and 0.86 for sustained virological responders to interferon +/- ribavirin treatment. The Health Utilities Index Mark 3 survey and the EuroQol Index survey utilities were lower than Canadian population norms (p < 0.001). Patient-elicited utilities were lower than previous expert estimates for mild/moderate chronic infection and sustained virological responders, but higher for decompensated cirrhosis and hepatocellular carcinoma. The Short Form-36v2 survey scores revealed several significant health impairments (p < 0.005) when compared with U.S. population norms. These findings 1) suggest that quality of life (QOL) differences across the HCV clinical spectrum are smaller than previously believed; 2) support other evidence suggesting that QOL is significantly diminished in HCV patients; and 3) provide utility values derived directly from HCV patients.

Health-state utilities and quality of life in hepatitis C patients

Objective Health-state utilities are global measurements of quality of life on a scale from 0 (death) to 1 (full health). Utilities are used to evaluate health outcomes and are the preferred outcome measure for policy models that determine the cost-effectiveness of treatments. Currently, utilities for hepatitis C virus (HCV)-infected patients have been estimated using expert judgments. The purpose of this study was to elicit HCV utilities directly from patients. Methods We assessed the utilities of 193 outpatients at various stages of chronic HCV progression by using a visual analog scale, the standard gamble technique, the Health Utilities Index Mark 3 survey, and the EuroQol Index survey. We also incorporated the nonutility-based Short Form-36v2 survey, which provides a detailed profile of health status. Results The mean standard gamble utilities were: 0.78 for patients without a recent liver biopsy and no signs of cirrhosis; 0.79 for mild to moderate chronic HCV infection; 0.80 for compensated cirrhosis; 0.60 for decompensated cirrhosis; 0.72 for hepatocellular carcinoma; 0.73 for transplant; and 0.86 for sustained virological responders to interferon ± ribavirin treatment. The Health Utilities Index Mark 3 survey and the EuroQol Index survey utilities were lower than Canadian population norms ( p < 0.001). Patient-elicited utilities were lower than previous expert estimates for mild/moderate chronic infection and sustained virological responders, but higher for decompensated cirrhosis and hepatocellular carcinoma. The Short Form-36v2 survey scores revealed several significant health impairments ( p < 0.005) when compared with U.S. population norms. Conclusions These findings 1) suggest that quality of life (QOL) differences across the HCV clinical spectrum are smaller than previously believed; 2) support other evidence suggesting that QOL is significantly diminished in HCV patients; and 3) provide utility values derived directly from HCV patients.