Idiopathic noncirrhotic portal hypertension Schouten, Jeoffrey N.L.; Garcia‐Pagan, Juan C.; Valla, Dominique C. ...
Hepatology,
2 September 2011, Letnik:
54, Številka:
3
Journal Article
Recenzirano
Odprti dostop
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. (HEPATOLOGY 2011;)
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.
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.
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. (Hepatology 2016;63:138–147)
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 AND AIMTo 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.
METHODSAll 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.
RESULTSIn 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 (OR2.97; 95% CI1.38–6.42; P=0.005) or two biopsy passes (OR1.87; 95% CI1.10–3.19; P=0.021). The risk of severe complications was not influenced by the number of biopsy passes (P>0.24). Hepatic malignancy (OR3.21; 95% CI1.18–8.73; P=0.022) and international normalized ratio 1.4 or more (OR7.03; 95% CI2.74–18.08; P<0.001) were risk factors of severe complications.
CONCLUSIONSevere 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.
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/m2 ) 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.
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.
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.
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