Nonobstructive Neonatal Cholestasis: Clinical Outcome and Scoring of the Histopathological Changes in Liver Biopsies

Abstract

The clinical outcome of nonobstructive neonatal cholestasis (NC) cases varies greatly and the prognosis is generally unpredictable. In this study, we aimed to evaluate the prognostic benefits of qualitative analysis of histopathological changes in nonobstructive NC cases. A total of 28 nonobstructive NC cases (18 neonatal hepatitis; 10 intrahepatic bile duct paucity) were studied. We analyzed the relationship between histopathological and clinical parameters. Hepatic inflammation, bridging necrosis, pericellular fibrosis, giant cell transformation, and extramedullary hematopoiesis were evaluated and scored according to their absence or presence in each case. The sum of the histopathological scores was accepted as "total pathological injury score." The height percentiles, the presence and the degree of hepatomegaly and ascites, and serum alanine aminotransferase (ALT), albumin, and bilirubin levels and prothrombin time were also evaluated and scored. The patients were divided into 2 clinical course groups considered "good" or "bad" according to the total clinical scores. For statistical analysis, Pearson's chi-square test, Mann-Whitney U-test, and receiver operating characteristic curve were used. We found a statistically significant negative relation between the clinical course and total pathological injury score (P = 0.042) and pericellular fibrosis (P = 0.016). In conclusion, during the interpretation of liver biopsies of nonobstructive NC, scoring of histopathological changes should be done for assessing the clinical prognostic outcome.

Diagnóstico diferencial de colestase neonatal: parâmetros clínicos e laboratoriais

“…8,14,19,22,23 The median age of infants at the time of referral is a cause for concern. In the EHC group, age at referral was over 60 days (77 days) of life and higher than that in the IHC group.…”

“…14 For early identification of BA cases, the use of a stool color card, 3,15 ultrasonography for the presence of the triangular cord sign, 16,17 and also correlation of clinical, laboratory and histopathological findings have been proposed. 3,14,[18][19][20] This study is aimed at evaluating whether clinical and laboratory parameters may help to establish differentiation between IHC and EHC. Patients were divided into two groups: group I with IHC and group II with EHC.…”

To the editor

“…It would be ideal that liver biopsy is included as a routine procedure during the operation and complement with the fi nal diagnosis. [4,5] Author reply W e thank Dr. Yeh and Chang for their valuable comments on our paper. We agree with their view points, which are complementary to our study.…”

Can Neonatal Hepatitis Be More Fatal than Biliary Atresia?

“…The frequency of NC is difficult to evaluate with certainty, but varies between 1:500 and 1:5000 live births [1,2]. The initial approach in the diagnosis of NC is to distinguish extrahepatitic obstructive causes from non-obstructive intrahepatitic disorders [3].…”

“…Because of the similarity of the clinical presentation of NH and BA, many cases of NH are misdiagnosed as BA, resulting in unnecessary exploratory surgery [1][2][3][4][5]. On the other hand, misdiagnosing BA as NH may result in progressive cirrhosis.…”