Primary biliary cirrhosis among atomic bomb survivors in Nagasaki, Japan

Ohba, Kazuo; Omagari, Katsuhisa; Kinoshita, Hideki; Soda, Hiroshi; Masuda, Junichi; Hazama, Hiroaki; Tagawa, Masuko; Hata, Tomoko; Nakamura, Hideo; Murata, Ikuo; Kohno, Shigeru

doi:10.1016/s0895-4356(00)00370-x

Cited by 41 publications

(21 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Additionally, radiation may also be a risk factor influencing the clustering of PBC cases. Increased prevalence of PBC was reported among atomic bomb survivors in Nagasaki compared to the general population of Japan [77] . Further evidence is surely needed to validate these findings.…”

Section: Environmental Factorsmentioning

confidence: 94%

Geoepidemiology, Genetic and Environmental Risk Factors for PBC

Zhang

Carbone

Lleo

et al. 2015

Dig Dis

View full text Add to dashboard Cite

Primary biliary cirrhosis (PBC) is the most paradigmatic autoimmune liver disease with still several controversial issues in epidemiology, diagnosis, causation, and therapy. Although we are witnessing an enormous increase in the quantum of our basic knowledge of the disease with an initial translation in clinical practice, there are still a number of key open questions in PBC. Among them are the following questions: Why are there vast geographical variations in disease frequency? What are the reasons for female preponderance? Why do only small-size bile ducts get affected: What is the real role of genetics and epigenetics in its development? In particular, the prevalence of PBC is known to vary both on an international and a regional level, suggesting the existence of substantive geographical differences in terms of genetic susceptibility and environmental factors. New theories on potential environmental triggers, such as chemical xenobiotics, which lead to the breaking of self-tolerance within a unique immunological milieu of the liver, have been suggested. On the other hand, new and solid data on the genetic architecture of PBC are now obtained from recent high-throughput studies, together with data on sex chromosomes defects, and epigenetic abnormalities, thus strongly suggesting a role of genetic and epigenetic factors in the triggering and perpetuation of the autoimmune aggression in PBC. Based on these evidences, a number of novel drugs directed against specific immune-related molecules are currently under development. In this paper, we review a comprehensive collection of current epidemiological reports from various world regions. We also discuss here the most recent data regarding candidate genetic and environmental risk factors for PBC.

show abstract

Section: Environmental Factorsmentioning

confidence: 94%

Geoepidemiology, Genetic and Environmental Risk Factors for PBC

Zhang

Carbone

Lleo

et al. 2015

Dig Dis

View full text Add to dashboard Cite

show abstract

“…Assuming a life expectancy of 20 years after diagnosis, the point prevalence was estimated to be 207 per million, and for women above 45 years, 860 per million [12]. As in some other studies [13,14], the incidence of AMA positivity without evidence of liver disease was two times higher than the incidence of AMA positivity with evidence of liver disease. It has been observed that the incidence and prevalence rates are highest in Northern European countries (e.g., England and Scotland) [15,16] and in the Northern United States (e.g., Minnesota) [17].…”

Section: Epidemiologymentioning

confidence: 95%

Primary biliary cirrhosis: A 2010 update

Poupon

2010

Journal of Hepatology

252

206

View full text Add to dashboard Cite

Primary biliary cirrhosis (PBC) is a chronic inflammatory autoimmune disease that mainly targets the cholangiocytes of the interlobular bile ducts in the liver. The condition primarily affects middle-aged women. Without treatment, PBC generally progresses to cirrhosis and eventually liver failure over a period of 10-20 years. PBC is a rare disease with prevalence of less than 1/2000. PBC is thought to result from a combination of multiple genetic factors and superimposed environmental triggers. The contribution of the genetic predisposition is evidenced by the familial clustering. Several risk factors, including exposure to infectious agents and chemical xenobiotics, have been suggested. Ursodeoxycholic acid (UDCA) is currently the only FDA-approved medical treatment for PBC. When administered at doses of 13-15 mg/kg/day, a majority of patients with PBC have a normal life expectancy without additional therapeutic measures. One out of three patients does not adequately respond to UDCA therapy and may need additional medical therapy and/or liver transplantation. This review summarises current knowledge on the epidemiology, ethiopathogenesis, clinical, and therapeutic aspects of PBC.

show abstract

“…Prediction map identified areas of high risk (11 patients per 50 km 2 ) in the Eastern part, while low risk areas (122 patients per 50 km 2 ) were located in the Western part of the island [13] . It should be noted that all epidemiological estimations may represent only the tip of the iceberg since it has been reported and confirmed that the incidence of antimitochondrial antibody (AMA) positivity without liver disease is two times higher than the incidence of AMA positivity with liver disease [19,20] . The significance of these findings is unclear.…”

Section: Epidemiologymentioning

confidence: 99%

Primary biliary cirrhosis: From bench to bedside

Notas

2015

WJGPT

View full text Add to dashboard Cite

Primary biliary cirrhosis (PBC) is a chronic nonsuppurative destructive intrahepatic cholangitis leading to cirrhosis after a protractive non cirrhotic stage. The etiology and pathogenesis are largely unknown and autoimmne mechanisms have been implicated to explain the pathological lesions. Many epitopes and autoantigens have been reported as crucial in the pathophysiology of the disease and T and B cells abnormalities have been described, the exact pathways leading to the destruction of small intrahepatic ductules are mostly speculative. In this review we examined the various epidemiologal and geoepidemiological data as well as the complex pathogenetic aspects of this disease, focusing on recent in vivo and in vitro studies in this field. Initiation and progression of PBC is believed to be a multifactorial process with strong infuences from the patient's genetic background and by various environmental factors. The role of innate and adaptive immunity, including cytokines, chemokines, macrophages and the involvement of apoptosis and reactive oxygen species are outlined in detailed. The current pathogenetic aspects are presented and a novel pathogenetic theory unifying the accumulated clinical information with in vitro and in vivo data is formulated.A review of clinical manifestations and immunological and pathological diagnosis was presented. Treatment modalities, including the multiple mechanisms of action of ursodeoxycholate were finally discussed. Core tip: Primary biliary cirrhosis (PBC) is a chronic nonsuppurative destructive intrahepatic cholangitis leading to cirrhosis of largely unknown pathophysiology. We examined the epidemiological and geoepidemiological data as well as the pathogenetic aspects of PBC. A novel pathogenetic theory unifying the accumulated clinical information with in vitro and in vivo data is formulated.

show abstract

Primary biliary cirrhosis among atomic bomb survivors in Nagasaki, Japan

Cited by 41 publications

References 18 publications

Geoepidemiology, Genetic and Environmental Risk Factors for PBC

Geoepidemiology, Genetic and Environmental Risk Factors for PBC

Primary biliary cirrhosis: A 2010 update

Primary biliary cirrhosis: From bench to bedside

Contact Info

Product

Resources

About