Serum bilirubin: a prognostic factor in primary biliary cirrhosis.

Shapiro, Janet; Smith, Hamilton; Schaffner, Fenton

doi:10.1136/gut.20.2.137

Cited by 301 publications

(130 citation statements)

References 7 publications

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“…Elevation of serum bilirubin is a late phenomenon and an excellent predictor of survival. 3 As in any patient with chronic cholestasis, total serum cholesterol may be elevated. fluorescence (IF) using rat stomach and kidney.…”

Section: Biochemical Testsmentioning

confidence: 99%

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Management of primary biliary cirrhosis

2000

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Primary biliary cirrhosis (PBC) is a presumed autoimmune disease of the liver, which predominantly affects women once over the age of 20 years. Most cases are diagnosed when asymptomatic (60%). The antimitochondrial antibody is present in serum in most, but not in all, patients with PBC. The disease generally progresses slowly but survival is less than an age-and gender-matched general population. The symptomatic patient may have fatigue, generalized pruritus, portal hypertension, osteoporosis, skin xanthomata, fat soluble vitamin deficiencies, and/or recurrent asymptomatic urinary tract infections. Many nonhepatic autoimmune diseases are found in association with PBC and may prompt initial presentation. To date, immunosuppressive therapy has not been shown to prolong survival in PBC. The hydrophilic bile acid, ursodeoxycholic acid (UDCA), has been shown when given in a dose of 13 to 15 mg/kg daily for up to 4 years to delay the time to liver transplantation or death. This therapy also causes a significant improvement of all the biochemical markers of cholestasis but has no beneficial effects on any of the symptoms or associated disorders. Treatment with UDCA does not obviate the need for liver transplantation. Therapies to prevent complications arising from malabsorption, portal hypertension, and/or osteoporosis are required as well. Good control of pruritus can be achieved in most patients. PBC is diagnosed with increasing frequency, but the agent(s) responsible for this slowly progressive destruction of the interlobular bile ducts remains elusive and hence a specific therapy remains unavailable. (HEPATOLOGY 2000;31:1005-1013.) PREAMBLE

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Section: Biochemical Testsmentioning

confidence: 99%

“…The most reliable determinants of prognosis in PBC are the height of the serum bilirubin 3 and the Mayo risk score. 80 The Mayo risk score is calculated as R ϭ 0.871 log e (bilirubin in mg/dL) ϩ Ϫ2.53 log e (albumin in g/dL) ϩ 0.039 age in years ϩ 2.38 log e (prothrombin time in seconds) ϩ 0.859(edema score of 0, 0.5, or 1).…”

Section: Liver Transplantationmentioning

confidence: 99%

Management of primary biliary cirrhosis

2000

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“…Bilirubin has been consistently demonstrated to be the single most important serum marker of survival [36][37][38] and therefore is a desirable endpoint. The limiting factor of using bilirubin as an endpoint is that it only becomes abnormal in late stages of the disease and thus is not a sensitive outcome measure.…”

Section: Biochemical Markersmentioning

confidence: 99%

“…Of all the serum markers studied to date, serum bilirubin is the best independent predictor of survival. [36][37][38] Table 1 lists several of the more compelling features upon which to consider subject stratification. Determining which ones are important enough to use for stratification of subjects in a clinical trial depends on the endpoint being examined.…”

Section: Stratification Of Subjectsmentioning

confidence: 99%

American association for the study of liver diseases endpoints conference: Design and endpoints for clinical trials in primary biliary cirrhosis

et al. 2010

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PreambleA group of multidisciplinary experts on primary biliary cirrhosis (PBC) and its complications convened on May 31, 2009, under the aegis of the American Association for the Study of Liver Diseases (AASLD) in order to identify the most appropriate design and endpoints for clinical trials of PBC based on current evidence and expert experience. The natural history of PBC was reviewed as well as current therapies. The current approaches to evaluating therapies for disease progression and symptoms as priorities were discussed. Appropriate aspects of trial design including entry criteria, study duration, and appropriate handling of issues such as stratification of subjects and use of ursodeoxycholic acid (UDCA), were identified and discussed. After a full day of presentations, in a consensus manner, appropriate endpoints for clinical efficacy trials regarding PBC and its complications were agreed upon and are reported in this summary.

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“…5 In untreated patients with PBC, elevated levels of serum bilirubin level (SBL) have been consistently found to be an independent predictor of a poor prognosis. [6][7][8][9] UDCA therapy substantially lowers SBL but has not been shown to consistently improve clinical and histological parameters. [1][2][3][4] Nevertheless, SBL is one of the criteria applied in the decision for referral to OLT.…”

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confidence: 99%

Clinical significance of serum bilirubin levels under ursodeoxycholic acid therapy in patients with primary biliary cirrhosis

Bonnand

et al. 1999

Hepatology

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We determined whether the normalization of serum bilirubin level (SBL) induced by ursodeoxycholic acid (UDCA) therapy was associated with an improved clinical outcome in patients with primary biliary cirrhosis (PBC). We estimated the prognostic values of SBL measured after 6 months of UDCA treatment for survival free of orthotopic liver transplantation (OLT). We used a database of 548 patients with PBC followed in three trials of UDCA. Among UDCA-treated patients, we compared survival free of OLT in patients with normalized SBL (I17 mol/L) with those who had persistently elevated SBL. Difference in survival was tested between UDCA-treated patients whose SBL normalized with treatment and placebo patients who had normal baseline SBL. We evaluated, in each treatment group, the prognostic value of 6-month SBL. Survival was estimated using the Kaplan-Meier method and compared by the Cox model. Survival free of OLT was significantly longer in patients who had normalized SBL (P F .0001; relative risk [RR]: 3.7, UDCA group). Survival free of OLT was not significantly different between UDCA patients with normalized SBL and placebo patients with a normal baseline SBL (P ‫؍‬ .69). For several cutoffs of 6-month SBL, RRs of OLT or death were similar in UDCA-treated and placebo patients: the RR of OLT or death associated with a 6-month SBL more than 30 mol/L was 6.0 for UDCA and 5.7 for placebo groups. In conclusion, normalization of SBL during therapy is associated with improved clinical outcome. SBL under UDCA therapy is a prognostic factor in PBC. SBL under UDCA therapy should be interpreted as in untreated patients. (HEPATOLOGY 1999;29:39-43.)

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Serum bilirubin: a prognostic factor in primary biliary cirrhosis.

Cited by 301 publications

References 7 publications

Management of primary biliary cirrhosis

Management of primary biliary cirrhosis

American association for the study of liver diseases endpoints conference: Design and endpoints for clinical trials in primary biliary cirrhosis

Clinical significance of serum bilirubin levels under ursodeoxycholic acid therapy in patients with primary biliary cirrhosis

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