Efficacy of Liver Transplantation in Patients with Primary Biliary Cirrhosis

Markus, B. H.; Dickson, E. Rolland; Grambsch, Patricia M.; Fleming, Thomas R.; Mazzaferro, Vincenzo; Klintmalm, Göran B.; Wiesner, Russell H.; Thiel, David H. Van; Starzl, Thomas E.

doi:10.1056/nejm198906293202602

Cited by 218 publications

(77 citation statements)

References 9 publications

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“…This analysis showed a 58% gain in survival at 1 yr in the highest-risk patients (average preoperative bilirubin, 28 mg/dl) , 55% in the intermediate group (bilirubin, 24 mg/dl) but only 14% in the best-risk cohort (bilirubin, 12 mg/dl) , whose predicted l-yr survival without transplantation had been 69%. The degree of rehabilitation of the survivors and the death rate after 1 yr were the same no matter how sick the patients were at surgery (18 Similar trends were reported in good-, intermediateand high-risk patients with the diagnosis of sclerosing cholangitis (20) whose actual posttransplant outcomes were compared with those predicted with a second Mayo prediction model (21) derived from age, bilirubin, splenomegaly and histopathological stage. The percent gain in survival of best-risk patients in the first year compared with that in their surrogate controls was a modest 7%; even after 7 yr, the percentage difference was still only 7% (60% vs. 53%).…”

Section: Discussionsupporting

confidence: 57%

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Disease gravity and urgency of need as guidelines for liver allocation*1

EGHTESAD¹

1994

Hepatology

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One thousand one hundred and twenty-eight candidates for liver transplantation were stratified into five urgency-of-need categories by the United Network for Organ Sharing (UNOS) criteria. Most patients of low-risk UNOS 1 status remained alive after 1 yr without transplantation; the mortality while waiting was 3% after a median of 229_5 days. In contrast, only 3% of those entered at the highest risk UNOS 5 category survived without transplantation; 28% died while waiting, the deaths occurring at a median of 5.5 days. The UN08 categories in between showed the expected gradations, in which at each higher level fewer patients remained as candidates throughout the l-yr duration of study while progressively more died at earlier and earlier times while waiting for an organ. In a separate study of posttransplantation survival during the same time period, the best postoperative results were in the lowest-risk UN08 1 and 2 patients (88% combined), and the worst results were those in UNOS 5 (71%). However, a relative risk cross-analysis showed that a negative benefit of transplantation may have been the result in terms of l-yr survival for the low-risk elective patients, but that a gain in life extension was achieved in the potentially lethal UNOS categories 3, 4 and 5 (greatest for UN08 3). These findings and conclusions are discussed in terms of total care of patients with liver disease, and in the context of organ allocation policies of the United States and Europe. (HEPA-TOLOGY 1994;20:56S-628.) Many of the earlier presentations at this Consensus Development Conference have focused on specific diseases and the wisdom of performing liver replacement for disorders that are apt to recur (such as cancer, hepatitis and alcoholic cirrhosis). Others have discussed limiting the operation to prime-of-life candidates, with exclusion of the very young or aged. Expansion of the donor pool has been proposed in the "splitting" oflivers

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Section: Discussionsupporting

confidence: 57%

“…One such study was of patients with PBC treated by transplantation from March 1980 through June 1987 at the universities of Colorado and Pittsburgh (18). The cases were studied retrospectively by physicians at the Mayo Clinic who independently stratified the patients into three categories -low, midrange and high.…”

Section: Discussionmentioning

confidence: 99%

Disease gravity and urgency of need as guidelines for liver allocation*1

EGHTESAD¹

1994

Hepatology

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“…7 The survival benefit of transplantation is evident as soon as 3 months after surgery, with 2-year survival of patients who underwent transplantation more than twice that predicted for those treated conservatively. 9 These results, independently confirmed by other groups, provide the most compelling evidence that liver transplantation improves survival among patients with chronic liver disease. 7 PSC.…”

Section: Pbcmentioning

confidence: 65%

Liver transplantation

Carithers

2000

Liver Transpl

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Liver transplantation has revolutionized the care of patients with end-stage liver disease. Liver transplantation is indicated for acute or chronic liver failure from any cause. Because there are no randomized controlled trials of liver transplantation versus no therapy, the efficacy of this surgery is best assessed by carefully comparing postoperative survival with the known natural history of the disease in question. The best examples of this are in primary biliary cirrhosis and primary sclerosing cholangitis, for which well-validated disease-specific models of natural history are available. There are currently relatively few absolute contraindications to liver transplantation. These include severe cardiopulmonary disease, uncontrolled systemic infection, extrahepatic malignancy, severe psychiatric or neurological disorders, and absence of a viable splanchnic venous inflow system. One of the most frequently encountered contraindications to transplantation is ongoing destructive behavior caused by drug and alcohol addiction. The timing of the surgery can have a profound impact on the mortality and morbidity of patients undergoing liver transplantation. Because of the long waiting lists for donor organs, the need to project far in advance when transplantation might be required has proven to be one of the greatest challenges to those treating patients with end-stage liver disease. Three important questions must be addressed in a patient being considered for liver transplantation: (1) when should the patient be referred for possible transplantation? (2) when should the patient be listed for transplantation? and (3) when is the patient too sick to have a reasonable chance of surviving the perioperative period?

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“…2 In 1989, we reported on the efficacy of orthotopic liver transplantation demonstrating that in patients with end-stage PBC, actual patient survival following transplantation was far superior to the expected survival without transplantation as predicted by the Mayo natural history model. 3 In the same analysis, pretransplantation disease severity, as measured by the risk score, had a significant impact on mortality posttransplantation, particularly in the first 90 days.…”

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confidence: 90%

Optimal timing of liver transplantation for primary biliary cirrhosis

et al. 1998

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In 1989, we reported on the efficacy of liver transplantation in primary biliary cirrhosis (PBC) by demonstrating that the actual patient survival following transplantation was significantly better than without transplantation as predicted by a mathematical survival model (''Mayo natural history model''). Our aim in this investigation was to determine an optimal time to perform liver transplantation in PBC. One hundred forty-three patients with PBC undergoing liver transplantation were followed prospectively. Disease severity was measured immediately before transplantation by a summary score (''risk score'') used in the Mayo natural history model, namely age, bilirubin, albumin, prothrombin time, and the presence or absence of edema. Proportional hazards analyses were performed assessing patient survival following transplantation. The influence of disease severity immediately pretransplantation on resource utilization for liver transplantation was assessed. Compared with our report in 1989, liver transplantation was performed at an earlier stage of disease (e.g., median risk score: 7.5 vs. 8.3; P F .01). Following transplantation, patient survival probabilities at 1, 2, and 5 years were 93%, 90%, and 88%, respectively. In the proportional hazards analysis, the risk of death following transplantation remained low until reaching a risk score of 7.8. In contrast, risk scores greater than 7.8 were associated with a progressively increased mortality. Resource utilization measured by the days in the intensive care unit (ICU) and hospital and the requirement for intraoperative blood transfusions was significantly greater in recipients who had higher risk scores before transplantation. Our data suggest that an optimal timing for liver transplantation, as determined by patient survival and resource utilization, appears to be at a risk score around 7.8 in patients with PBC. (HEPATOLOGY 1998;28:33-38.)

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Efficacy of Liver Transplantation in Patients with Primary Biliary Cirrhosis

Cited by 218 publications

References 9 publications

Disease gravity and urgency of need as guidelines for liver allocation*1

Disease gravity and urgency of need as guidelines for liver allocation*1

Liver transplantation

Optimal timing of liver transplantation for primary biliary cirrhosis

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