Primary biliary cirrhosis

Primary biliary cirrhosis (PBC) is a chronic and slowly progressive cholestatic liver disease of autoimmune etiology characterized by injury of the intrahepatic bile ducts that may eventually lead to liver failure. Affected individuals are usually in their fifth to seventh decades of life at time of diagnosis, and 90% are women. Annual incidence is estimated between 0.7 and 49 cases per million-population and prevalence between 6.7 and 940 cases per million-population (depending on age and sex). The majority of patients are asymptomatic at diagnosis, however, some patients present with symptoms of fatigue and/or pruritus. Patients may even present with ascites, hepatic encephalopathy and/or esophageal variceal hemorrhage. PBC is associated with other autoimmune diseases such as Sjogren's syndrome, scleroderma, Raynaud's phenomenon and CREST syndrome and is regarded as an organ specific autoimmune disease. Genetic susceptibility as a predisposing factor for PBC has been suggested. Environmental factors may have potential causative role (infection, chemicals, smoking). Diagnosis is based on a combination of clinical features, abnormal liver biochemical pattern in a cholestatic picture persisting for more than six months and presence of detectable antimitochondrial antibodies (AMA) in serum. All AMA negative patients with cholestatic liver disease should be carefully evaluated with cholangiography and liver biopsy. Ursodeoxycholic acid (UDCA) is the only currently known medication that can slow the disease progression. Patients, particularly those who start UDCA treatment at early-stage disease and who respond in terms of improvement of the liver biochemistry, have a good prognosis. Liver transplantation is usually an option for patients with liver failure and the outcome is 70% survival at 7 years. Recently, animal models have been discovered that may provide a new insight into the pathogenesis of this disease and facilitate appreciation for novel treatment in PBC

Primary biliary cirrhosis, demonstrating chronic non-suppurative destructive cholangitis (stage 1 of Scheuer's classification)

Infiltration of lymphocyte () and plasma cell () into bile duct is shown. (D-PAS.)<p><b>Copyright information:</b></p><p>Taken from "Primary biliary cirrhosis"</p><p>http://www.ojrd.com/content/3/1/1</p><p>Orphanet Journal of Rare Diseases 2008;3():1-1.</p><p>Published online 23 Jan 2008</p><p>PMCID:PMC2266722.</p><p></p

Alcohol Consumption is Positively Associated with Handgrip Strength Among Japanese Community-dwelling Middle-aged and Elderly Persons

Summary: Background: Alcohol consumption is an important lifestyle factor for a variety of health problems, we investigated whether alcohol consumption is associated with handgrip strength (HGS), which is a useful indicator of sarcopenia, among Japanese community-dwelling persons. Methods: The present study included 764 men aged 70 (69–70) years and 955 women aged 70 (69–70) years from a rural village. Daily alcohol consumption was measured using the Japanese liquor unit in which a unit corresponds to 22.9 g of ethanol, and the participants were classified into never drinkers, occasional drinkers, daily light drinkers (1–2 units/day), and daily moderate drinkers (2–3 units/day). Results: HGS were significantly correlated with age in both men and women. HGS increased significantly with increased daily alcohol consumption in both genders, and in men HGS in daily moderate drinkers were significantly greater than those in never, occasional, and daily light drinkers. In women, HGS in daily light and moderate drinkers were significantly greater than those in never drinkers. In men, Multivariate-adjusted HGS were significantly greater in daily light {mean: 33.4 (95% confidence interval: 32.3–34.5) kg} and moderate drinkers {33.6 (32.8–34.0) kg} than in never drinkers {31.7 (30.8–32.7) kg}, and in women multivariate-adjusted HGS in occasional drinkers {21.5 (21.0–22.1) kg} was significantly greater in never drinkers {20.7 (20.5–21.0) kg}. Conclusion: These results suggest that alcohol consumption may have a protective role in aging-associated decline in muscle strength in community-dwelling persons. Keywords: alcohol consumption, handgrip strength, aging, confounding facto

Thigh-hip ratio is significantly associated with all-cause mortality among Japanese community-dwelling men.

Anthropometric evaluation is a simple yet essential indicator of muscle and fat mass when studying life prognosis in aging. This study aimed to investigate the contributions of anthropometric measurements, independent of body mass index, to measures of all-cause mortality. We examined data for 1,704 participants from the 2014 Nomura Cohort Study who attended follow-ups for the subsequent eight years (follow-up rate: 93.0%). Of these, 765 were male (aged 69 ± 11 years) and 939 were female (aged 69 ± 9 years). The Japanese Basic Resident Registry provided data on adjusted relative hazards for all-cause mortality. The data were subjected to a Cox regression analysis, wherein the time variable was age and the risk factors were gender, age, anthropometric index, smoking habits, drinking habits, exercise habits, cardiovascular history, hypertension, lipid levels, diabetes, renal function, and serum uric acid. Of the total number of participants, 158 (9.3%) were confirmed to have died, and of these, 92 were male (12.0% of all male participants) and 66 were female (7.0% of all female participants). The multivariable Cox regression analysis revealed that a smaller thigh-hip ratio predicted eight-year all-cause mortality in male participants, but only baseline body mass index was associated with all-cause mortality in female participants. Thigh-hip ratio is a useful predictor of death in Japanese community-dwelling men

Infection as a Risk Factor in the Pathogenesis of Primary Biliary Cirrhosis: Pros and Cons

Primary biliary cirrhosis (PBC) is a chronic and slowly progressive cholestatic liver disease of autoimmune etiology, characterized by injury of the intrahepatic bile ducts that may eventually lead to cirrhosis and liver failure. Evidence suggests cardinal roles for both environmental factors and genetic susceptibility. Nevertheless, the absolute etiology of PBC is unclear, despite recent well-designed case-control studies that reported environmental risk factors, including infectious agents, for PBC. Of the reported infectious agents, some of them are not reproducible and remain controversial. However, infection is no doubt one of the major risks among the environmental factors. This is supported by the fact that infectious agents in autoimmune diseases express antigens resulting in molecular mimicry and xenobiotics that play a role in breaking tolerance. Taken together, recent findings from genome wide assays as well as novel animal models may enable us to better understand the mechanism of pathogenesis responsible for this disease

Primary biliary cirrhosis, showing ductular proliferation (stage 2 of Scheuer's classification)

Small ductular structures () is shown in a portal tract (). (Hematoxylin-eosin.)<p><b>Copyright information:</b></p><p>Taken from "Primary biliary cirrhosis"</p><p>http://www.ojrd.com/content/3/1/1</p><p>Orphanet Journal of Rare Diseases 2008;3():1-1.</p><p>Published online 23 Jan 2008</p><p>PMCID:PMC2266722.</p><p></p

Mildly elevated serum total bilirubin is negatively associated with hemoglobin A1c independently of confounding factors among community-dwelling middle-aged and elderly persons

Abnormally high glycated hemoglobin (Hb) (HbA1c) is significantly associated with oxidative stress and an increased risk of cardiovascular disease (CVD). Serum total bilirubin (T-B) may have a beneficial role in preventing oxidative changes and be a negative risk factor of CVD. Limited information is available on whether serum T-B is an independent confounding factor of HbA1c. The study subjects were 633 men aged 70 ± 9 (mean ± standard deviation (SD)) years and 878 women aged 70 ± 8 years who were enrolled consecutively from among patients aged ≥40 years through a community-based annual check-up process. We evaluated the relationship between various confounding factors including serum T-B and HbA1c in each gender. Multiple linear regression analysis pertaining to HbA1c showed that in men, serum T-B ( β = −0.139) as well as waist circumference ( β = 0.099), exercise habit ( β = 0.137), systolic blood pressure (SBP) ( β = 0.076), triglycerides ( β = 0.087), and uric acid ( β = −0.123) were significantly and independently associated with HbA1c, and in women, serum T-B ( β = −0.084) as well as body mass index ( β = 0.090), smoking status ( β = −0.077), SBP ( β = 0.117), diastolic blood pressure (DBP) ( β = −0.155), low-density lipoprotein cholesterol ( β = 0.074), prevalence of antidyslipidemic medication ( β = 0.174), and uric acid ( β = 0.090) were also significantly and independently associated with HbA1c. Multivariate-adjusted serum HbA1c levels were significantly high in subjects with the lowest serum T-B levels in both genders. Serum T-B is an independent confounding factor for HbA1c among community-dwelling middle-aged and elderly persons

Usefulness of waist-to-height ratio in screening incident metabolic syndrome among Japanese community-dwelling elderly individuals.

This study examined a range of anthropometric indices and their relationships with metabolic syndrome (MetS). Despite recommendations that central obesity assessment should be employed as a marker of metabolic health, there is no consensus regarding the protocol for measurement. The present study included 720 men aged 71 ± 8 years and 919 women aged 71 ± 7 years from a rural village. We examined the relationship between anthropometric indices {e.g., body mass index (BMI), waist-to-height ratio (WHtR), waist-to-hip ratio (WHpR)}, and MetS based on the modified criteria of the National Cholesterol Education Program's Adult Treatment Panel (NCEP-ATP) III report in a cross-sectional (N = 1,639) and cohort (N = 377) data. A receiver operating curve (ROC) analysis was performed to determine the optimal cut-off value and best discriminatory value of each of these anthropometric indices to predict MetS. In the cross-sectional study, WHtR as well as BMI and WHpR showed significantly predictive abilities for MetS in both genders; and WHtR showed the strongest predictive ability for the presence of MetS. Also in the cohort study, WHtR as well as BMI and WHpR showed significantly predictive abilities for incident MetS in both genders, and in men WHtR showed the strongest predictive ability for incident MetS, but in women BMI showed the strongest predictive ability. In the cross-sectional study, the optimal WHtR cutoff values were 0.52 (sensitivity, 71.0%; specificity, 77.9%) for men and 0.53 (sensitivity, 79.8%; specificity, 75.7%) for women. In the cohort study, the optimal WHtR values were 0.50 (sensitivity, 60.7%; specificity, 73.2%) for men and 0.50 (sensitivity, 75.0%; specificity, 56.1%) for women. Increased WHtR was significantly and independently associated with prevalence of MetS in both genders. These results suggest that WHtR is a useful screening tool for determining metabolic risk in Japanese elderly community dwelling individuals