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Indication and Assessment for Liver Transplantation CME Liver disease 14 Rossle M, Ochs A, Gulberg V, Siegerstetter V, et al. A comparison of paracentesis and Indication and assessment for transjugular intrahepatic portosystemic shunting in patients with ascites. N Engl J liver transplantation Med 2000;342:1701–7. 15 Lake JR. The role of transjugular shunting in patients with ascites. N Engl J Med 2000; 342:1745–7. Paul B Southern MB ChB MRCP , used. The Child-Pugh classification 2 Transplant Fellow (Table1) allows objective assessment of a Mervyn H Davies MD FRCP, patient’s functional liver status and in the Lead Clinician in Hepatology USA forms the basis for the criteria Department of Medical Hepatology, required to list patients. Those with St James’s University Hospital, Leeds Childs C grade have a 58%, 21% and 0% one-year, five-year and 10-year survival, Clin Med JRCPL 2002;2:313–6 respectively. Subjective measures of liver disease may be more difficult to assess. Tools are Liver transplantation provides effective available to document quality of life 3, therapy for most forms of acute and and a full psychosocial assessment chronic liver failure – one-year survival should be carried out. rates exceed 90% 1 – and the indications continue to expand. In general terms, the Cholestatic liver disease indications for liver transplantation are objective evidence of liver failure and Primary biliary cirrhosis subjective criteria such as poor quality of Primary biliary cirrhosis (PBC) is a life due to liver disease and occasionally declining indication for liver transplan- rare metabolic defects. tation, but still accounted for 7.8% of liver transplants in Europe in 1998– Chronic liver disease 20001. The disease has three stages: an initial asymptomatic stage In chronic liver disease the most impor- tant aspect of patient selection is timing. a symptomatic stage with worsening Transplantation should improve both cholestasis and declining synthetic quality and quantity of life. The proce- function (falling albumin and dure is optimally carried out when the increasing prothrombin time) patient is well enough to withstand the a decompensating stage with severe procedure, but ill enough to warrant it jaundice and evidence of portal (iepredicted survival is about 1–2 years hypertension. without a transplant). The natural history of PBC is well Assessment for transplantation in defined. Various prognostic models have chronic liver disease is difficult. been designed. The most commonly Objective and subjective measures are used is the Mayo Clinic model 4; this has Key Points There are objective and subjective criteria for transplantation Disease-specific criteria exist for different conditions Certain indications are common to all conditions, for example: failing synthetic function (serum albumin <25 g/l) complications of portal hypertension intolerable quality of life Discussion/referral should occur prior to end-stage disease KEY WORDS: acute liver disease, chronic liver disease, CPD, liver transplantation Clinical Medicine Vol 2No 4 July/August 2002 313 CME Liver disease allowed prediction of estimated survival Table 1. Childs grading of severity in chronic liver disease with Pugh’s 1973 of PBC patients with and without trans- modifications. plantation. Criteria assessed Points scored for increasing abnormality Bilirubin may be used as an indicator 1 2 3 for transplantation. Serum bilirubin levels of 100 µmol/l and 150 µmol/l give Encephalopathy grade None I–II III–IV median survivals of 24 months and Ascites Absent Slight Moderate 17 months, respectively. Treatment with ursodeoxycholic acid has meant that Serum bilirubin (µmol/ l): <35 35–50 >50 patients are less likely to be transplanted In PBC <70 70–170 >170 as a result of their serum bilirubin, but Serum albumin (g/l) >35 35–28 <28 more because of resistant ascites, other complications of portal hypertension Prothrombin time (prolongation in sec): 1–4 4–10 >10 (egvariceal bleed) or declining synthetic function. Occasionally, patients with Total score <6 A 7–9 B >10 C PBC are transplanted because of PBC = primary biliary cirrhosis. intractable pruritus or severe lethargy. Survival for PBC after transplantation is 80–90% at 10 years, with a low risk (<10%) of symptomatic PBC recurrence. Autoimmune hepatitis alcohol contract’ prior to listing. The Patients should be referred for transplan- six-month period of abstinence is impor- tation if their expected survival is less Autoimmune hepatitis (AIH) usually tant since some patients may recover than twoyears, but before carries a good prognosis, with 93% during the time, thus negating the need they begin to decompensate rapidly or five-year survival. Several features sug- for a transplant. subjective features such as lethargy gest a bad prognosis, such as onset in Assessment of patients with alcoholic or pruritus make their quality of life childhood, typeII disease (liver-kidney liver disease includes investigations to intolerable. microsomal antibody (LKM)-positive) rule out any comorbid alcohol-related and failure to respond to immunosup- illnesses such as cardiomyopathy, nutri- pressive treatment (associated with a tional failure or nephropathy. In patients Primary sclerosing cholangitis 6 69% mortality at four years) . with alcoholic liver disease, cirrhosis is Models exist for the prognosis of primary Patients with AIH may present with often a late development. The onset of sclerosing cholangitis (PSC) and actuarial acute liver failure. It may be possible to ascites reduces median survival to survival post transplant, using these induce remission with appropriate 12 months, and the development of models is 89% at five years, compared immunosuppression, but this should be spontaneous bacterial peritonitis sug- with 31% in medically managed performed in a transplant centre so that, gests a median survival of six months. patients5. The variables used to predict should treatment fail, emergency trans- Hepatocellular carcinoma (HCC) will be survival are serum bilirubin, haemo- plantation can be provided. developed by 20% of patients with alco- globin, histological state on liver biopsy, Transplantation for AIH has a good holic cirrhosis, and 75% of patients will age and presence of inflammatory bowel prognosis (80% five-year survival). Graft die from their liver disease. disease. failure due to disease recurrence is rare. The indications for liver transplanta- Referral for transplantation is usually for tion in patients with PSC include: chronic liver failure when predicted Viral hepatitis survival is less than 1–2 years. severe jaundice (bilirubin Hepatitis B >100 µmol/l) Hepatitis B is a common cause of complications arising from portal Alcoholic cirrhosis cirrhosis worldwide, but less so in the hypertension, and Alcoholic cirrhosis is the second com- UK. The outcome of transplantation for poor quality of life. monest indication for liver transplanta- hepatitis B was universally poor in the There is a high rate of cholangiocarci- tion, accounting for 20% of European early days of transplantation due to graft noma, with some studies suggesting a liver transplants. reinfection and the rapid onset of liver prevalence of 30% at 10 years. Un- When assessing a patient, non-depen- failure. It is mandatory that patients with diagnosed cholangiocarcinoma is found dent alcohol abuse should be differenti- end-stage hepatitisB virus (HBV) infec- in 8–18% of explanted livers, although ated from alcoholism. Most transplant tion are rendered HBV DNA negative incidental cholangiocarcinoma, either programmes in the UK insist on a with antiviral agents such as lamivudine microscopic or smaller than 1cm, does six-month abstinence period prior to prior to transplantation. Recurrence of not necessarily affect prognosis. transplantation and the signing of a ‘no hepatitis B after transplantation is pre- 314 Clinical Medicine Vol 2No 4 July/August 2002 CME Liver disease vented by administration of hepatitis B has been achieved by tightening the successful, but the outlook is not so good immunoglobulin. criteria for transplantation: a single once cirrhosis has developed. Patients Five-year survival for patients with lesion of less than 5cm or less than may decompensate or develop HCC, compensated HBV cirrhosis (ie Childs A fivelesions in total and no evidence especially if alcohol misuse is a cofactor. disease) is 85%, and for decompensated of vascular or capsular invasion. Alpha-1-antitrypsin is another inherited disease (Childs C) is between 14 and Transplantation outside these guidelines disorder for which transplantation is 35%7. Indications for transplantation in should occur only as part of a clinical indicated when a patient has decompen- HBV are objective criteria of liver failure, trial. sated cirrhosis. In this situation, the with declining serum albumin usually outlook is excellent and soon after the best early indicator. Concerns over Metabolic indications transplant the patient expresses the graft reinfection mean that patients with alpha-1-antitrypsin phenotype of the subjective symptoms are managed There are several metabolic indications donor organ. medically. for liver transplantation (Table2). Wilson’s disease can present both as Other indications Hepatitis C chronic liver disease and as acute liver failure. In the acute situation, patients Many other indications exist for trans- Hepatitis C is the commonest indication invariably die without liver transplanta- plantation. Transplantation may be used for transplantation worldwide. Of those tion. Genetic haemachromatosis is the
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