scholarly journals Twelve-Year-Old Girl with Primary Biliary Cirrhosis

2012 ◽  
Vol 2012 ◽  
pp. 1-3 ◽  
Author(s):  
Ivana Kitic ◽  
Aleksandra Boskovic ◽  
Ivica Stankovic ◽  
Dragan Prokic
Keyword(s):  
Primary Biliary Cirrhosis ◽  
World Literature ◽  
Pediatric Patients ◽  
Immunoglobulin M ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis ◽  
Antimitochondrial Antibodies ◽  
Biochemical Tests ◽  
Autoimmune Etiology

Primary biliary cirrhosis (PBC) is a slowly progressive cholestatic liver disease of autoimmune etiology. The initial presentation of PBC is varies from asymptomatic, abnormal liver biochemical tests to overt cirrhosis. Unlike other autoimmune liver diseases, PBC has rarely been reported in childhood. We report a case of primary biliary cirrhosis in a 12-year-old girl. In addition to characteristic histology features, strongly positive antimitochondrial antibodies, increased liver enzyme levels, increased serum quantitative immunoglobulin M levels, and cholestasis were discovered. She had been treated with ursodeoxycholic acid. In the world literature, we found only few pediatric patients of primary biliary cirrhosis. Aetiology, pathogenesis, the long-term natural history, and prognosis remain obscure. Due to increased awareness of early-onset PBC, rather than typical older ones, further pediatric cases may be discovered.

scholarly journals Rheumatoid Arthritis and Primary Biliary Cirrhosis: Cause, Consequence, or Coincidence?

Arthritis ◽  
2012 ◽  
Vol 2012 ◽  
pp. 1-7 ◽  
Author(s):  
Daniel S. Smyk ◽  
Dimitrios P. Bogdanos ◽  
Maria G. Mytilinaiou ◽  
Andrew K. Burroughs ◽  
Eirini I. Rigopoulou
Keyword(s):  
Rheumatoid Arthritis ◽  
Primary Biliary Cirrhosis ◽  
High Titre ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis ◽  
Infectious Agents ◽  
Antimitochondrial Antibodies ◽  
Genetic Studies ◽  
Genetic Traits ◽  
Large Case

Primary biliary cirrhosis (PBC) is a progressive cholestatic liver disease characterized serologically by cholestasis and the presence of high-titre antimitochondrial antibodies and histologically by chronic nonsuppurative cholangitis and granulomata. PBC patients often have concomitant autoimmune diseases, including arthropathies. This raises the question as to whether there are shared features in the pathogenesis of those diseases with the pathogenesis of PBC. Epidemiological and large case studies have indicated that although the incidence of rheumatoid arthritis (RA) is not significantly raised in PBC patients, there appears to be a higher rate of RA in PBC patients and their relatives. Genetic studies have demonstrated that several genes implicated in PBC have also been implicated in RA. Epigenetic studies provided a wealth of data regarding RA, but the findings on epigenetic changes in PBC are very limited. As well, certain infectious agents identified in the pathogenesis of PBC may also play a role in the pathogenesis of RA. These data suggest that although RA is not significantly present in PBC, some individuals with certain genetic traits and environmental exposures may develop both conditions. This concept may also apply to other concomitant diseases found in PBC patients.

scholarly journals Oral Tolerance and Pyruvate Dehydrogenase in Patients with Primary Biliary Cirrhosis

2002 ◽  
Vol 9 (2) ◽  
pp. 55-61 ◽  
Author(s):  
Ayako Suzuki ◽  
Judy van de Water ◽  
M. Eric Gershwin ◽  
Roberta Jorgensen ◽  
Paul Angulo ◽  
...  
Keyword(s):  
Primary Biliary Cirrhosis ◽  
Pyruvate Dehydrogenase ◽  
Oral Tolerance ◽  
Early Stage ◽  
Pyruvate Dehydrogenase Complex ◽  
Tolerance Induction ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis ◽  
Antimitochondrial Antibodies ◽  
Immunological Destruction

Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease characterized by the immunological destruction of intralobular bile ducts and serum anti-mitochondrial antibodies (AMA). Based upon previous work of oral tolerance and autoimmunity, we hypothesized that feeding the mitochondrial autoantigens of PBC would alter the clinical course and the level of antimitochondrial antibodies. The bovine pyruvate dehydrogenase complex (PDC) was purified and 5 mg fed in gelatin capsules to 6 patients with early stage PBC for 6 months. Antimitochondrial antibodies and liver biochemistries were measured at every 3 months for 12 months. The clinical trial was completed for all patients except for 1 who showed deterioration of pre-existing skin rash during treatment, which disappeared within 2 weeks after treatment was discontinued. However, after 1 year, neither the titers of AMAs nor liver biochemistries were significantly changed by this treatment. This is the first trial to test the efficacy of oral tolerance induction in PBC. However, the data, which limited in scope, did not demonstrate efficacy and further highlights the difficulties in showing continuing evidence of tolerance induction in autoimmunity.

scholarly journals Sex Differences Associated with Primary Biliary Cirrhosis

2012 ◽  
Vol 2012 ◽  
pp. 1-11 ◽  
Author(s):  
Daniel S. Smyk ◽  
Eirini I. Rigopoulou ◽  
Albert Pares ◽  
Charalambos Billinis ◽  
Andrew K. Burroughs ◽  
...  
Keyword(s):  
Primary Biliary Cirrhosis ◽  
Clinical Course ◽  
Epidemiological Studies ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis ◽  
Antimitochondrial Antibodies ◽  
Intrahepatic Bile Ducts ◽  
Life Threatening ◽  
Low Incidence ◽  
Disease Specific

Primary biliary cirrhosis (PBC) is a cholestatic liver disease of autoimmune origin, characterised by the destruction of small intrahepatic bile ducts. The disease has an unpredictable clinical course but may progress to fibrosis and cirrhosis. The diagnostic hallmark of PBC is the presence of disease-specific antimitochondrial antibodies (AMA), which are pathognomonic for the development of PBC. The disease overwhelmingly affects females, with some cases of male PBC being reported. The reasons underlying the low incidence of males with PBC are largely unknown. Epidemiological studies estimate that approximately 7–11% of PBC patients are males. There does not appear to be any histological, serological, or biochemical differences between male and female PBC, although the symptomatology may differ, with males being at higher risk of life-threatening complications such as gastrointestinal bleeding and hepatoma. Studies on X chromosome and sex hormones are of interest when studying the low preponderance of PBC in males; however, these studies are far from conclusive. This paper will critically analyze the literature surrounding PBC in males.

Therapy of Primary Biliary Cirrhosis: Novel Approaches for Patients with Suboptimal Response to Ursodeoxycholic Acid

2015 ◽  
Vol 33 (Suppl. 2) ◽  
pp. 125-133 ◽  
Author(s):  
Albert Parés
Keyword(s):  
Bile Acid ◽  
Primary Biliary Cirrhosis ◽  
Ursodeoxycholic Acid ◽  
Combined Treatment ◽  
Immunoglobulin M ◽  
Biochemical Response ◽  
Biliary Cirrhosis ◽  
Long Term Outcome ◽  
Increased Risk

Primary biliary cirrhosis (PBC) is a chronic cholestatic disease of presumed autoimmune pathogenesis, characterized by the inflammation and damage of the intrahepatic intermediate and small bile ducts, which eventually results in cirrhosis. A number of randomized and observational and pilot studies using several agents were carried out in the 80s, but no clear results or even harmful effects were reported. Over the past 2 decades, increasing evidence indicates that ursodeoxycholic acid (UDCA) - 13 to 16 mg/kg/day - is the treatment of choice for patients with PBC. Biochemical response to UDCA, assessed at 1 year, clearly predicts the long-term outcome, since in UDCA, responders survival is similar to that estimated for the matched control population. However, about 40% of patients have incomplete biochemical response and increased risk of progression and decreased survival free of transplantation. Patients with suboptimal biochemical response to UDCA outline the group in whom further single or combined treatments with UDCA are needed. Accordingly, data on the effect of fibrates alone or in combination with UDCA, and budesonide in combination with UDCA have been reported. The combined treatment of UDCA and fibrates in patients without optimal biochemical response to UDCA improves the degree of cholestasis and may minimize the long-term management of these patients. The results of the combined therapy of UDCA with budesonide are appealing but they should be established in large randomized trials. The effect of new agents such obeticholic acid are promising, since the addition of this farnesoide-X-receptor agonist bile acid in patients with stable UDCA dosage and increased alkaline phosphatase levels results in an improvement of cholestasis as compared to placebo, with a parallel decrease of aminotransferases and immunoglobulin M, as well as one surrogate marker of bile acid synthesis. New molecular therapies are currently being investigated.

Clincal effect of long-term (32 weeks) eicosapentate administation in primary biliary cirrhosis

2001 ◽  
Vol 120 (5) ◽  
pp. A355-A355
Author(s):  
K TAKAGI ◽  
M TAKAHASHI ◽  
H TAKADA ◽  
Y KITAZUMI ◽  
T ARIMA ◽  
...  
Keyword(s):  
Primary Biliary Cirrhosis ◽  
Biliary Cirrhosis

A novel etiologic factor of highly elevated cholestanol levels: progressive familial intrahepatic cholestasis

2020 ◽  
Vol 33 (5) ◽  
pp. 665-669
Author(s):  
Aynur Küçükçongar Yavaş ◽  
Büşra Çavdarlı ◽  
Özlem Ünal Uzun ◽  
Ayşen Uncuoğlu ◽  
Mehmet Gündüz
Keyword(s):  
Primary Biliary Cirrhosis ◽  
Intrahepatic Cholestasis ◽  
Density Lipoprotein ◽  
Etiologic Factor ◽  
Compound Heterozygous ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis ◽  
Progressive Familial Intrahepatic Cholestasis ◽  
Turkish Patient ◽  
Type 3

AbstractBackgroundProgressive familial intrahepatic cholestasis type 3 (PFIC3) is an uncommon cholestatic liver disease caused by mutations in the ATP binding cassette subfamily B member 4 (ABCB4) gene. Although PFIC3 is frequently identified in childhood, ABCB4 disease-causing alleles have been described in adults affected by intrahepatic cholestasis of pregnancy, hormone-induced cholestasis, low-phospholipid-associated cholelithiasis syndrome or juvenile cholelithiasis, cholangiocarcinoma and in sporadic forms of primary biliary cirrhosis. Cholestanol is a biomarker which is elevated especially in cerebrotendinous xanthomatosis and rarely in primary biliary cirrhosis (PBC) and Niemann Pick type C.Case presentationHere we report a Turkish patient with compound heterozygous mutations in the ABCB4 gene, who has hepatosplenomegaly, low level of high-density lipoprotein, cholestasis and high level of cholestanol.ConclusionThis is the first PFIC3 case with a high cholestanol level described in the literature. There are very few diseases linked to increased cholestanol levels, two of which are CTX and PBC. From this case, we can conclude that a high cholestanol level might be another indicator of PFIC type 3.

scholarly journals Comment on biochemical response to ursodeoxycholic acid and long-term prognosis in primary biliary cirrhosis

Hepatology ◽  
2008 ◽  
Vol 49 (1) ◽  
pp. 337-338 ◽  
Author(s):  
Lucy J. Walker ◽  
Julia Newton ◽  
David E. J. Jones ◽  
Margaret F. Bassendine
Keyword(s):  
Primary Biliary Cirrhosis ◽  
Ursodeoxycholic Acid ◽  
Biochemical Response ◽  
Biliary Cirrhosis ◽  
Long Term Prognosis

Effects of long-term rifampicin administration in primary biliary cirrhosis

1989 ◽  
Vol 9 ◽  
pp. S105
Author(s):  
L. Bachs ◽  
A. Parés ◽  
M. Elena ◽  
C. Piera ◽  
J. Rodés
Keyword(s):  
Primary Biliary Cirrhosis ◽  
Biliary Cirrhosis

scholarly journals A sensitive bead assay for antimitochondrial antibodies: Chipping away at AMA-negative primary biliary cirrhosis

Hepatology ◽  
2007 ◽  
Vol 45 (3) ◽  
pp. 659-665 ◽  
Author(s):  
Sabine Oertelt ◽  
Roman Rieger ◽  
Carlo Selmi ◽  
Pietro Invernizzi ◽  
Aftab A. Ansari ◽  
...  
Keyword(s):  
Primary Biliary Cirrhosis ◽  
Biliary Cirrhosis ◽  
Antimitochondrial Antibodies
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