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Background and Aim: To minimize the sample variability of liver biopsy, the tissue length should be at least 25 mm. Consequently, more than one biopsy pass is needed with cutting biopsy needles. We aimed to investigate the risk factors of biopsy-related complication, including the number of biopsy passes., Methods: All consecutive liver biopsies performed between 2005 and 2014 were included. Biopsies were ultrasound assisted and performed with cutting biopsy needles. A complication was an event where the patient visited a healthcare provider because of biopsy-related complaints. Complications followed by hospitalization 2 or more days or intervention were considered severe., Results: In total, 1806 liver biopsies were analyzed. Overall, 102 (5.6%) complications were observed, of which 31 (1.7%) were severe. One (0.06%) patient died. Common complications were pain (n=75/102; 74%) and bleeding (n=34/102; 33%). Two biopsy passes were not associated with an increased risk of complications compared with one biopsy pass [odds ratio (OR): 1.59; 95% confidence interval (CI): 0.83-3.04; P=0.16], whereas three or more biopsy passes increased this risk compared with one (OR: 2.97; 95% CI: 1.38-6.42; P=0.005) or two biopsy passes (OR: 1.87; 95% CI: 1.10-3.19; P=0.021). The risk of severe complications was not influenced by the number of biopsy passes (P>0.24). Hepatic malignancy (OR: 3.21; 95% CI: 1.18-8.73; P=0.022) and international normalized ratio 1.4 or more (OR: 7.03; 95% CI: 2.74-18.08; P<0.001) were risk factors of severe complications., Conclusion: Severe complication rate and mortality were low. Performing multiple biopsy passes was not associated with severe complications, whereas hepatic malignancy or elevated international normalized ratio were associated with an increased risk.

Prevalence of non-alcoholic fatty liver disease is increased in patients with psoriasis. However, it is not known how liver fibrosis correlates with psoriasis. This study investigated the association between psoriasis and liver fibrosis compared with participants without psoriasis within the population-based Rotterdam Study. All participants were screened for liver fibrosis using transient elastography. Liver stiffness > 9.5 kPa suggested advanced liver fibrosis. Psoriasis was identified using a validated algorithm. A total of 1,535 participants were included (mean age ± standard deviation 70.5 ± 7.9 years; 50.8% female; median body mass index 26.4 kg/m2 (interquartile range 24.2-28.9)) of whom 74 (4.7%) had psoriasis. Prevalence of advanced liver fibrosis was 8.1% in psoriasis patients compared with 3.6% in the reference group (p = 0.05). The risk of advanced liver fibrosis in psoriasis patients remained comparable after adjustment for demographics, lifestyle characteristics and laboratory findings (odds ratio 2.57 (95% confidence interval 1.00-6.63). This study suggests that elderly people with psoriasis are twice as likely to have advanced liver fibrosis irrespective of common risk factors.

Unlabelled: Given that little is known about the prevalence of, and factors associated with, liver fibrosis in the general population, we aimed to investigate this in a large, well-characterized cohort by means of transient elastography (TE). This study was part of the Rotterdam Study, a population-based study among individuals ≥45 years. All participants underwent abdominal ultrasound and TE. Liver stiffness measurement (LSM) ≥8.0 kilopascals (kPa) was used as a cutoff suggesting clinically relevant fibrosis. Of 3,041 participants (age, 66.0 ± 7.6 years) with reliable LSM, 169 (5.6%) participants had LSM ≥8.0 kPa. Age (odds ratio [OR]: 2.40; 95% confidence interval [CI]: 1.72-3.36; P < 0.001), alanine aminotransferase (ALT; OR, 1.24; 95% CI: 1.12-1.38; P < 0.001), smoking (OR, 1.77; 95% CI: 1.16-2.70; P = 0.008), spleen size (OR, 1.23; 95% CI: 1.09-1.40; P = 0.001), hepatitis B surface antigen, or anti-hepatitis C virus positivity (OR, 5.38; 95% CI: 1.60-18.0; P = 0.006), and combined presence of diabetes mellitus (DM) and steatosis (OR, 5.20; 95% CI: 3.01-8.98; P < 0.001 for combined presence) were associated with LSM ≥8.0 kPa in multivariable analyses. The adjusted predicted probability of LSM ≥8.0 kPa increased per age decade, with probabilities ranging from 1.4% (0.9-3.6) in participants ages 50-60 years to 9.9% (6.8-14.5) in participants >80 years. Participants with both DM and steatosis had the highest probabilities of LSM ≥8.0 kPa (overall probability: 17.2% [12.5-23.4]; this probability did not increase with age [P = 0.8])., Conclusion: In this large population-based study of older adults, LSM ≥8.0 kPa, suggestive of clinically relevant fibrosis, was present in 5.6% and was strongly associated with steatosis and DM. In the context of an aging population and an increased prevalence of DM and obesity, this study illustrates that liver fibrosis may become a more prominent public health issue in the near future., (© 2015 by the American Association for the Study of Liver Diseases.)

Background & Aims: The coagulation system is known to be involved in fibrogenesis in patients with liver disease. We investigated whether common genetic prothrombotic risk factors are associated with an increased risk of fibrosis in the general population., Methods: This investigation was part of the Rotterdam Study, an ongoing, population-based cohort study. Liver stiffness (LS) was measured using transient elastography (Fibroscan) and associated with single nucleotide polymorphisms determining blood group type and presence of the Factor V Leiden (FVL) mutation or prothrombin G20210A gene variant., Results: Reliable LS measurements and genetic data were obtained from 1055 Caucasian participants. LS ⩾8.0 kPa, suggestive of clinically relevant fibrosis, was observed in 101 subjects (9.6%). Presence of FVL or prothrombin G20210A was independently associated with an increased risk of LS ⩾8.0 kPa (OR 2.09, 95%CI 1.07-4.07, p=0.03). Combination of blood group type non-O and the FVL mutation or prothrombin G20210A variant resulted in an even higher risk of LS ⩾8.0 kPa (OR 3.36, 95%CI 1.50-7.56, p=0.003). Presence of the FVL mutation or prothrombin G20210A variant in participants with blood group non-O was associated with a predicted probability of 14.3% (7.7-23.8) of LS ⩾8.0 kPa., Conclusions: Participants carrying the FVL mutation or prothrombin G20210A variant have an increased risk of clinically relevant liver fibrosis, which is even higher in blood group type non-O carriers. The fact that genetic prothrombotic risk factors are associated with an increased risk of liver fibrosis suggests that coagulation plays an important role in fibrogenesis in the general population., (Copyright © 2015 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.)

Idiopathic non-cirrhotic portal hypertension (INCPH) is a rare disease characterized of intrahepatic portal hypertension in the absence of cirrhosis or other causes of liver disease and splanchnic venous thrombosis. The etiology of INCPH can be classified in five categories: 1) immunological disorders (i.e. association with common variable immunodeficiency syndrome, connective tissue diseases, Crohn's disease, etc.), 2) chronic infections, 3) exposure to medications or toxins (e.g. azathioprine, 6- thioguanine, arsenic), 4) genetic predisposition (i.e. familial aggregation and association with Adams-Oliver syndrome and Turner disease) and 5) prothrombotic conditions (e.g. inherited thrombophilias myeloproliferative neoplasm antiphospholipid syndrome). Roughly, INCPH diagnosis is based on clinical criteria and the formal exclusion of any other causes of portal hypertension. A formal diagnosis is based on the following criteria: 1) presence of unequivocal signs of portal hypertension, 2) absence of cirrhosis, advanced fibrosis or other causes of chronic liver diseases, and 3) absence of thrombosis of the hepatic veins or of the portal vein at imaging. Patients with INCPH usually present with signs or symptoms of portal hypertension such as gastro-esophageal varices, variceal bleeding or splenomegaly. Ascites and/or liver failure can occur in the context of precipitating factors. The development of portal vein thrombosis is common. Survival is manly limited by concomitant disorders. Currently, treatment of INCPH relies on the prevention of complications related to portal hypertension, following current guidelines of cirrhotic portal hypertension. No treatment has been studied aimed to modify the natural history of the disease. Anticoagulation therapy can be considered in patients who develop portal vein thrombosis.

Background: Non-alcoholic fatty liver or hepatic steatosis is considered the hepatic manifestation of the metabolic syndrome. Statins are often used by patients with metabolic syndrome, but their effect in steatosis is not well established., Aims: To study the association between statins and the presence of steatosis., Methods: In the population-based Rotterdam Study, 2578 subjects underwent liver ultrasonography and had prescription data available. In a cross-sectional design, we investigated the effect of current, past, and duration of statin use. Logistic regression analyses were adjusted for age, sex, and other known risk factors., Results: The prevalence of steatosis was 35.3%. We identified 631 current and 359 past statin users. In multivariable analyses, current statin use >2 years was associated with a significantly lower steatosis prevalence [OR 0.43, 95% CI 0.19-0.96]. Stratification by mean body mass index showed that this association was stronger in patients with body mass index ≥ 27.5 [OR 0.30, 95% CI 0.11-0.81 for current use >2 years], while in patients with body mass index <27.5 the association was non-significant., Conclusion: Within the Rotterdam study, in patients with body mass index ≥ 27.5 current use of statins for >2 years was associated with a lower prevalence of steatosis., (Copyright © 2014 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.)

Background: Recent case-control studies observed an increased prevalence of nonalcoholic fatty liver disease (NAFLD) in patients with psoriasis, which is relevant in selecting optimal psoriasis treatment., Objective: We sought to compare the prevalence of NAFLD in people with psoriasis and those without psoriasis., Methods: This large prospective population-based cohort study (part of the Rotterdam Study) enrolled elderly participants (>55 years). NAFLD was diagnosed as fatty liver on ultrasonography in the absence of other liver diseases. Participants with psoriasis were identified using a validated algorithm. Multivariable logistic regression model was used to assess whether psoriasis was associated with NAFLD after adjusting for demographic, lifestyle characteristics, and laboratory findings., Results: In total, 2292 participants were included (mean age 76.2 ± 6.0 years; 58.7% female; mean body mass index 27.4 ± 4.2kg/m(2)) of whom 118 (5.1%) had psoriasis. The prevalence of NAFLD was 46.2% in patients with psoriasis compared with 33.3% for the reference group without psoriasis (P = .005). Psoriasis was significantly associated with NAFLD; after adjustment for alcohol consumption, pack-years and smoking status, presence of metabolic syndrome, and alanine aminotransferase, psoriasis remained a significant predictor of NAFLD (adjusted odds ratio 1.7, 95% confidence interval 1.1-2.6)., Limitations: This was a cross-sectional study., Conclusion: Elderly participants with psoriasis are 70% more likely to have NAFLD than those without psoriasis independent of common NAFLD risk factors., (Copyright © 2013 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

Background & Aims: Little is known about the association of serum liver enzymes with long-term outcome in the elderly. We sought to clarify the association of serum gamma-glutamyltransferase (GGT), alkaline phosphatase (ALP), alanine aminotransferase (ALT) and aspartate aminotransferase (AST) with all-cause and cause-specific mortality in an elderly population., Methods: This study was embedded in the Rotterdam Study, a large population-based cohort of persons aged 55 years or older. Cox-regression analyses were performed to examine the association of baseline serum GGT, ALP, and aminotransferase levels with mortality, adjusted for age, sex, education, smoking status, alcohol intake, hypertension, diabetes mellitus, body mass index and total cholesterol levels. Liver enzyme levels were categorized according to sample percentiles; levels <25th percentile were taken as a reference., Results: During a follow-up of up to 19.5 years, 2997 of 5186(57.8%) participants died: 672 participants died of causes related to cardiovascular diseases (CVD) and 703 participants died of cancer. All serum liver enzymes were associated with all-cause mortality (all P < 0.001). Moreover, GGT was associated with increased CVD mortality (P < 0.001), and ALP and AST with increased cancer-related mortality (P = 0.03 and P = 0.005 respectively). Participants with GGT and ALP in the top 5% had the highest risk for all-cause mortality (HR1.55; 95%CI 1.30-1.85 and HR1.49; 95%CI 1.25-1.78 respectively). AST and ALT <25th percentile were also associated with a higher risk of all-cause mortality., Conclusions: All serum liver enzymes were positively associated with long-term mortality in this elderly population. Why participants with low ALT and AST levels have higher risk of mortality remains to be elucidated., (© 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Although chronic hepatitis has a diverse aetiology, development of hepatic fibrosis is the universal outcome. Cirrhosis is considered to be the universal end-stage of chronic liver disease. Patients with cirrhosis are at increased risk for liver failure and hepatocellular carcinoma. Hepatic fibrosis has long been considered irreversible. However, evidence has accumulated to suggest hepatic fibrosis can regress. In various liver diseases, it has been demonstrated that effective treatment of the underlying cause can result in regression of hepatic fibrosis. Currently, this is the best therapeutic approach for treatment of hepatic fibrosis. In patients with cirrhosis eradication or suppression of the underlying cause of the damage has also been shown to result in regression of hepatic fibrosis, however, it remains unclear if all histological transformations in cirrhosis are reversible. Patients with cirrhosis in whom the underlying cause was effectively treated have a reduced risk of liver failure and hepatocellular carcinoma, as well as improved survival.

Background & Aims: We aimed to validate the fatty liver index (FLI), an algorithm that is based on waist circumference, body mass index, and levels of triglyceride and γ-glutamyltransferase. We calculated its ability to identify fatty liver disease from any cause or nonalcoholic fatty liver disease (NAFLD) in a large population of white elderly persons., Methods: We collected ultrasonography and FLI data from participants of the Rotterdam Study from February 2009 to February 2012; 2652 subjects (mean age, 76.3 ± 6.0 years) were interviewed and received a clinical examination that included abdominal ultrasound, analysis of blood samples during fasting, and anthropometric assessment. The ability of the FLI to detect (nonalcoholic) fatty liver was assessed by using area under the receiver operator characteristic (AUROC) curve analysis., Results: FLI score was associated with NAFLD in multivariable analysis (odds ratio, 1.05; 95% confidence interval [CI], 1.04-1.05; P < .001). FLI identified patients with NAFLD with an AUROC curve of 0.813 (95% CI, 0.797-0.830) and those with fatty liver from any cause with an AUROC curve of 0.807 (95% CI, 0.792-0.823)., Conclusions: The FLI (an algorithm that is based on waist circumference, body mass index, and levels of triglyceride and γ-glutamyltransferase) accurately identifies NAFLD, confirmed via ultrasonography, in a large, white, elderly population., (Published by Elsevier Inc.)

Purpose: Patients with liver cirrhosis scheduled for liver transplantation often present with a concurrent umbilical hernia. Optimal management of these patients is not clear. The objective of this study was to compare the outcomes of patients who underwent umbilical hernia correction during liver transplantation through a separate infra-umbilical incision with those who underwent correction through the same incision used to perform the liver transplantation., Methods: In the period between 1990 and 2011, all 27 patients with umbilical hernia and liver cirrhosis who underwent hernia correction during liver transplantation were identified in our hospital database. In 17 cases, umbilical hernia repair was performed through a separate infra-umbilical incision (separate incision group) and 10 were corrected from within the abdominal cavity without a separate incision (same incision group). Six patients died during follow-up; no deaths were attributable to intraoperative umbilical hernia repair. All 21 patients who were alive visited the outpatient clinic to detect recurrent umbilical hernia., Results: One recurrent umbilical hernia was diagnosed in the separate incision group (6 %) and four (40 %) in the same incision group (p = 0.047). Two patients in the same incision group required repair of the recurrent umbilical hernia; one of whom underwent emergency surgery for bowel incarceration. The one recurrent hernia in the separate incision group was corrected electively., Conclusion: In the event of liver transplantation, umbilical hernia repair through a separate infra-umbilical incision is preferred over correction through the same incision used to perform the transplantation.

Aims: In the western world, idiopathic non-cirrhotic portal hypertension (INCPH) is a rare disease. This study aimed to investigate the histopathological features in western INCPH patients and to assess pathological differences between liver specimens of INCPH with and without HIV., Methods and Results: Biopsies of 70 INCPH patients (of which 15 were HIV-infected) were compared to 23 patients with non-cirrhotic portal vein thrombosis (PVT), which served as a control group for non-cirrhotic portal hypertension. Phlebosclerosis, nodular regeneration (NR), sinusoidal dilatation, paraportal shunting vessels, perisinusoidal fibrosis and portal tract remnants were the most prevalent morphological features of INCPH. There were significant (P < 0.01) morphological differences between INCPH and PVT liver specimens with regard to portal tract remnants (46% versus 0%), phlebosclerosis (95% versus 65%), portal vein dilatation (34% versus 78%) and NR (56% versus 22%). The degree of NR correlated with the severity of phlebosclerosis (P < 0.01). NR was seen more frequently in the HIV-INCPH group, compared to the non-HIV-infected patients (P < 0.001)., Conclusion: Portal tract remnants, phlebosclerosis and nodular regeneration are typical features of INCPH. Sinusoidal dilatation, paraportal shunting vessels and increased portal and parenchymal vessels might represent pressure-related morphological signs of portal hypertension. Finally, more nodular regeneration was observed in HIV-associated INCPH., (© 2013 Blackwell Publishing Ltd.)

Anticoagulant therapy is a cornerstone in the treatment of different liver diseases. In Budd-Chiari syndrome (BCS), survival rates have increased considerably since the introduction of a treatment strategy in which anticoagulation is the treatment of first choice. In all patients diagnosed with acute portal vein thrombosis (PVT), anticoagulant therapy for at least 3 months is indicated. Anticoagulation should also be considered in patients with chronic PVT and a concurrent prothrombotic risk factor. Current evidence suggests that patients with PVT in cirrhosis will benefit from treatment with anticoagulation as well. In severe chronic liver disease the levels of both pro- and anticoagulant factors are decreased, resetting the coagulant balance in an individual patient and making it more prone to deviate to a hypo- or hypercoagulable state. An increased activity of the coagulation cascade is not solely a feature of chronic liver disease; it influences the development of liver fibrosis as well. Several studies in animals and humans have shown that anticoagulation could prevent or improve fibrogenesis and even disease progression in cirrhosis. Anticoagulation is therefore a promising antifibrotic treatment modality.

Background & Aims: The prevalence of non-alcoholic fatty liver disease (NAFLD) appears to increase with age. However, limited data are available concerning the prevalence of NAFLD in the elderly. Our aim was to determine the prevalence and risk factors of NAFLD in an elderly population., Methods: This study was based on participants in the population-based Rotterdam Study. Each participant was interviewed and had a clinical examination at the research center, including a fasting blood collection, liver ultrasonography, and anthropometric assessment. Ordinal and logistic regression analysis was used to assess associations between covariables and (severity of) NAFLD., Results: Data from 2811 participants (mean age 76.4 ± 6.0 years) were analyzed. The prevalence of NAFLD was 35.1%. The prevalence of NAFLD decreased with advancing age (p<0.001). In logistic regression analysis, age (OR 0.97; 95% CI 0.95-0.99; p<0.001), total physical activity level (OR 0.98, 95% CI 0.96-0.99; p=0.005), pack years of smoking (OR 1.01, 95% CI 1.00-1.01; p=0.02), waist circumference >88 cm for women and > 102 cm for men (OR 4.89; CI 4.00-5.96; p<0.001), fasting glucose ≥ 100 mg/dl or drug treatment for elevated blood glucose (OR 2.11, 95% CI 1.72-2.59; p<0.001), blood pressure ≥ 130/85 mmHg or drug treatment for elevated blood pressure (OR 1.80, 95% CI 1.08-3.01; p=0.03), and triglycerides ≥ 150 mg/dl or treatment with serum lipid reducing agents (OR 1.56, 95% CI 1.28-1.91; p<0.001) were associated with NAFLD., Conclusions: NAFLD is common in the elderly, although the prevalence decreases with advancing age. Further studies are warranted exploring potential factors contributing to this apparent positive selection effect in the elderly., (Copyright © 2012 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.)

Background: Idiopathic noncirrhotic portal hypertension (INCPH) has been reported increasingly in patients with HIV infection., Aim: To evaluate the number of nationwide diagnosed HIV-associated INCPH cases and to assess its clinical features, risk factors and outcome., Methods: All HIV centres in the Netherlands were contacted and requested to notify INCPH cases diagnosed in their population. A case–control study was performed to identify the risk factors of INCPH. The cases were group-matched for duration of follow-up after HIV diagnosis to controls. Controls were selected from a database of HIV patients with negative screening for signs of portal hypertension on abdominal ultrasound. Univariate and multivariate conditional logistic regression analyses were performed., Results: On 1st of July 2011, 18.085 individuals were infected with HIV in the Netherlands. Within this population, sixteen patients with clinically overt INCPH were identified. At the time of INCPH diagnosis, cases had a lower platelet count and a higher ALT level. In univariate and multivariate analyses, didanosine [OR: 1.9 (1.3–2.8)], concomitant didanosine and stavudine treatment [OR: 6.3 (2.1–19.1)] and concomitant didanosine and tenofovir treatment [OR: 5.1 (1.2–22.6)] were independently associated INCPH. During follow-up, 4 patients died [malignancy (n = 3), liver failure (n = 1)]. A significant decline in platelets was observed after didanosine discontinuation (P = 0.003)., Conclusions: HIV-associated clinically relevant idiopathic noncirrhotic portal hypertension appears to be a rarely diagnosed disease. Long-term exposure to didanosine and short-term combination of didanosine and stavudine or tenofovir exposure are associated with idiopathic noncirrhotic portal hypertension. Mortality in HIV-associated idiopathic noncirrhotic portal hypertension is mainly related to HIV-associated disorders. Portal hypertension continues despite didanosine discontinuation

Background: Idiopathic noncirrhotic portal hypertension (INCPH) is a rare disease in the Western world. As a result, little is known about the clinical characteristics and outcome of these patients. Survival in these patients is considered to be similar to that of the general population., Aim: To investigate the clinical manifestations, pathophysiology, outcome and determinants of survival in Western INCPH patients., Methods: Multicentre cohort study of INCPH patients., Results: A total of 62 patients were followed for a median time of 90 months (range 24-310). Initial manifestations leading to the diagnosis of INCPH were related to portal hypertension in 82% of the patients. Histological signs of portal blood supply disturbances were present in nearly all patients. During follow-up, 12 of 62 patients developed liver decompensation, of which four were considered for liver transplantation. One patient died in the context of variceal bleeding. Hepatocellular carcinoma was not observed during follow-up. A total of 23 patients died during follow-up, only four of them due to liver related mortality. The Kaplan-Meier estimates for overall survival were 100% (95% CI 95-100%), 78% (95% CI 67-89%) and 56% (95% CI 40-72%) at 1, 5 and 10 years respectively. Survival for INCPH was significantly decreased (P < 0.001) compared to survival of the general population. Ascites was an independent predictor of poor outcome., Conclusions: In comparison to the general population, survival in INCPH patients is poor. Mortality is related to associated disorders and medical conditions occurring at older age. Patients rarely die due to liver related complications. Patients with ascites have a poor prognosis., (© 2012 Blackwell Publishing Ltd.)

Background: Liver allocation in Eurotransplant (ET) is based on the MELD score. Interlaboratory MELD score differences in INR and creatinine determination have been reported. The clinical implication of this observation has not been demonstrated., Methods: MELD scores were calculated in 66 patients with liver cirrhosis using bilirubin, creatinine, and INR analyzed in six liver transplant centers. Based on allocation results of ET, patients transplanted from December 2006 to June 2007 were divided according to MELD score in four groups. For each group, the influence of the match MELD on the probability of receiving a transplant was studied (Cox proportional hazards model)., Results: Laboratory-dependent significant differences in MELD score were demonstrated. Cox proportional hazards model showed a significant association between MELD score and the probability of organ allocation. The unadjusted hazard ratio for receiving a liver transplant was significantly different between group 2 and group 4 (group 2: MELD 19-24; group 4: MELD > 30)., Conclusion: Laboratory-dependent significant differences in MELD score were observed between the six transplant centers. We demonstrated a significant association between the MELD score and the probability of organ allocation. The observed interlaboratory variation might yield a significant difference in organ allocation in patients with high MELD scores., (© 2011 John Wiley & Sons A/S.)

Idiopathic noncirrhotic portal hypertension (INCPH) is characterized by an increased portal venous pressure gradient in the absence of a known cause of liver disease and portal vein thrombosis. In contrast to the high prevalence of this disorder in India, INCPH is a rare disease in the Western world. The etiology of INCPH can be divided in five categories: chronic infections, exposure to medication or toxins, thrombophilia, immunological disorders, and genetic disorders. Multifactorial etiology can also be encountered. Chronic abdominal infection is incriminated as the most important etiological factor in Eastern patients and thrombophilia in Western patients. The majority of patients with INCPH initially present with signs or complications of portal hypertension (mainly variceal bleeding and splenomegaly). These patients usually have preserved liver function. Liver function impairment occurs mainly in the context of intercurrent conditions. Patients with INCPH are often clinically and radiologically misdiagnosed as liver cirrhosis, so that a liver biopsy is indispensable to discriminate cirrhosis from INCPH. Histopathological characteristics of INCPH are heterogeneous, demonstrating overlap between several pathological entities (e.g., hepatoportal sclerosis, nodular regenerative hyperplasia, and incomplete septal cirrhosis). Even though hemodynamical changes in INCPH patients are not comparable to those in cirrhotics, prophylaxis and treatment of variceal bleeding are recommended to be similar. Anticoagulation therapy must be considered only in patients who develop portal vein thrombosis. INCPH has been considered a disorder with a relatively benign disease course. However, liver failure, hepatic encephalopathy, and hepatopulmonary syndrome can occur and are considered indications for liver transplantation., (Copyright © 2011 American Association for the Study of Liver Diseases.)

In three patients, two men aged 57 and 53 years, and a 43-year-old woman, idiopathic portal hypertension, also called non-cirrhotic portal hypertension (NCPH), was diagnosed. The first two patients presented with haematemesis. They were treated by endoscopic rubber band ligation of oesophageal varices. In the second patient, placement of a transjugular intrahepatic portosystemic shunt (TIPS) was necessary due to failure of the ligation treatment. The third patient was treated for HIV infection and had a gastroscopy because of nausea and vomiting, which revealed oesophageal varicosis. None of the patients had liver function impairment. Two of the patients had been treated with medication known to be associated with NCPH (azathioprine for Crohn's disease (second patient) and didanosine for HIV infection (third patient)). These medications were discontinued. The histological features of the patients were heterogeneous (nodular regenerative hyperplasia, periportal fibrosis and periportal dilated structures), but consistent with NCPH. Portal hypertension in the Western world is mostly associated with liver cirrhosis. When portal hypertension occurs in association with patent portal and hepatic veins, and in the absence of liver cirrhosis, NCPH must be considered. The prognosis of this disease is much better than that of cirrhosis.