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Copyright ©2008 The WJG Press and Baishideng.
World J Gastroenterol. Jun 7, 2008; 14(21): 3313-3327
Published online Jun 7, 2008. doi: 10.3748/wjg.14.3313
Table 1 Epidemiology of primary biliary cirrhosis: Results from the most relevant studies[29]
AreaPatients (No.)Prevalence (per million)Incidence (per million/yr)Age (yr)Gender (M:F)
Europe (1984)5692354541:10
Northern Sweden (1990)111151113.3551:6
North East England (1990)347129119581:9
Ontario, Canada (1990)225223.3591:13
Victoria, Australia (1995)8419--1:11
Newcastle, England (1997)160240122661:10
Olmsted County, MN (2000)46402127-1:8
Victoria, Australia (2004)249511-611:9
Table 2 Modifications during time of the clinical spectrum of primary biliary cirrhosis at presentation[5052]
Sherlock 1973James 1981Nyberg 1989
(n = 100)(n = 93)(n = 80)
Jaundice (%)28163
Pruritus (%)571426
Complications (%)491
Asymptomatics (%)116170
Mean age (yr)505758
Table 3 Parameters independently associated with bad prognosis in different prognostic models based on a single point observation[16113119121123]
Increase in serum bilirubin++++++
Decrease in serum albumin+++
Increase in PT (INR)+
Advanced age+++++
Ascites, fluid retention+++
Esophageal varices+
Gastrointestinal bleeding++
Cholestatic picture at histology++
Mallory bodies+
Table 4 Pharmacological characteristics of the opiate antagonists investigated in clinical studies
Pharmacological characteristics
NaloxoneVery short half life
Intravenous continuous infusion
Dose: 0.2-0.4 &mgr;g/kg per minute
NalmefeneLonger half life
Oral administration
2 mg twice/d with a gradual increase until 20 mg twice/d
NaltrexoneLonger half life
Oral administration
50 mg/d
(in two divided doses the first day and subsequently in a unique dose)
Table 5 Clinical management of metabolic bone disease associated with primary biliary cirrhosis
Clinical managementEfficacy
Moderate efficacyMild efficacy, insufficient data
1 Parenteral vitamin D3 supplementationIndicated for all patients to prevent osteomalacic lesions
2 Calcium carbonate supplementation
1 EstrogenFew data but effective and safe
2 EtidronateConflicting data
Indicated in case of concomitant corticosteroid administration
3 AlendronateFew data but effective and safe
4 CalcitoninProbably ineffective
Table 6 Efficacy and toxicity of the principal drugs investigated for the medical treatment of primary biliary cirrhosis
Table 7 Randomized, double-blind, placebo-controlled trials on ursodeoxycholic acid administration to patients with primary biliary cirrhosis
First authorNo. of patientsStudy design and duration of follow upUDCA effects on
Poupon[191]1462 yrImprovedImprovedNo effect
Heathcote[192]2222 yrNo effectImprovedNo effect
Lindor[193]180Mean follow up: 2 yrNo effectNo effectNo effect
Combes[194]1512 yrImprovedImproved (early stages)No effect
Eriksson[195]1162 yr + 2 yr as open trial (UDCA)No effectNo effectNo effect
Pares[196]192Mean follow up: 3.4 yrImprovedImprovedNo effect
Papatheodoritis[197]86Mean follow up 7.3 yr for UDCA 8.1 yr for controlsNot evaluatedNo effectNo effect