Usefulness of a scoring system in the interpretation ofhistology in neonatal cholestasis

Abstract

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, portoportal 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 ≥ 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 ≥ 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.

Hepatic expression of interferon-γ and CD68 in biliary atresia compared with other neonatal cholestatic disorders

“…These results are in agreement with those of Lee and Looi [9], who reported that histological interpretation of liver biopsies based on a seven criteria scoring system had the highest sensitivity (100%), specificity (75.9%), and diagnostic accuracy to differentiate BA from other intrahepatic cholestasis. Receiver operating characteristic curve for CD68 and interferon-g (IFN-g) in biliary atresia (BA).…”

“…This is in accordance with the findings of Rastogi et al [4], who reported that ductular proliferation was the most important histopathological feature to distinguish BA from other NC disorders. In addition, Cauduro [15], Lee and Looi [9], and Russo et al [10], reported that ductular proliferation had the best performance among the liver biopsy criteria for diagnosing BA.…”

“…Liver tissue histology was evaluated for the presence of portal ductular proliferation, bile plugs in bile ducts and ductules, as well as multinucleated giant cell transformation of hepatocytes. A 15-point scoring system based on the histology of liver biopsy, consisting of seven histological criteria developed by Lee and Looi [9], was applied for scoring BA. Portal fibrosis was graded as described in the study by Russo et al [10].…”

Can Neonatal Hepatitis Be More Fatal than Biliary Atresia?

“…Therefore, The North American Society for Pediatric Gastroenterology, Hepatology and Nutrition guideline recommends percutaneous liver biopsy in infants with undiagnosed NC [4,[8][9][10]. Accuracy of histopathological diagnosis has been reported in a broad range (60%-95%) because there are no certain histological criteria for differential diagnosis of NC [4,[11][12][13][14][15][16]. In addition, the characteristic morphologic changes of biliary atresia may not appear in the first month of life.…”

Cilostazol attenuates cholestatic liver injury and its complications in common bile duct ligated rats

“…Grading and scoring of ductal proliferation. Sections were assessed for the ductular proliferation score, as adapted from Lee and Looi, 2009. Bile ductular proliferation was considered to be present if the average number of ductules in the portal tract was more than five.…”