Liver transplantation has been successfully used in the treatment of a large number of liver diseases. The largest patient group comprises patients with end stage decompensated liver disease. Decompensation is defined as the presence of cirrhosis and one or more of the following: jaundice, ascites, hepatic encephalopathy, hepatorenal syndrome or bleeding oesophageal varices. In general patients in this category should be considered for liver transplantation, if available. Guidelines for liver transplant assessment have been published by both the British Society of Gastroenterology and the American Association for the Study of Liver Disease. These guidelines provide a good basis for patient selection. As new information becomes available the indications for individual diseases may change somewhat. One of the most important changes in recent years was the introduction of the MELD/PELD scoring system. This is the model for end stage liver disease which provides a reasonably robust estimate of prognosis for individual patients. Prior to this patient waiting time on the transplant list was one of the principal determinants of priority for liver allocation. The MELD scoring system has been widely adopted with the aim of allocating the available livers to patients in the greatest clinical need.
AIM: To ascertain the usefulness of a histological scoring system devised to assist in the interpretation of liver histology in neonatal cholestasis (NC).
METHODS: Liver biopsy specimens obtained from infants with NC referred to a tertiary pediatric unit in Malaysia were prospectively studied. The first author, blinded to the final diagnosis, devised the histological diagnosis based on a 7-feature (portal ductal proliferation, bile plugs in portal ductules, porto-portal bridging, lymphocytic infiltration in portal region, multinucleated hepatocytes, neutrophilic infiltration, hepatocellular swelling), 15-point (0 to 15) scoring system. The author classified the histological diagnosis as either biliary atresia (BA) or neonatal hepatitis (NH, all other diagnoses), and subsequently compared the author's diagnosis with the final diagnosis.
RESULTS: Eighty-four biopsy specimens obtained from 78 patients were reviewed. Without the scoring system, BA was correctly diagnosed by the author histologically in 30 cases, labelled as NH in 3. For other diagnoses, BA was excluded correctly in 33 cases and mislabeled as BA in 2 cases. The overall sensitivity for BA was 91%, specificity 86% and accuracy 88%. With the scoring system, a score of >or=7 had the best diagnostic utility to differentiate BA from other intrahepatic cholestasis histologically (sensitivity 88%, specificity 94%, accuracy 92%). Four patients with a score<7 had BA, and 3 patients with a score>or=7 had NH.
CONCLUSION: A 7-feature, 15-point histological scoring system had good diagnostic accuracy in the interpretation of liver histology in neonatal cholestasis.