Clinical features of congenital hepatic ifbrosis in children / 临床儿科杂志

Objective To explore the clinical features of congenital hepatic fibrosis (CHF) in children.MethodsA total of 111 patients diagnosed with CHF during January 2002 to June 2015 were included and divided into children group and adult group according to the age at diagnosis. The clinical data including gender, age, clinical symptoms, signs, laboratory examinations, and imaging characteristics were retrospectively analyzed and compared between two groups.ResultsThere were high incidences of splenomegaly and liver cirrhosis in both children and adult groups, and no difference was found between two groups (P>?0.05). The fatigue, jaundice, and hepatosplenomegaly were more common in children group while the renal cyst and liver cyst were more common in adult group, and the differences were statistically signiifcant (P all?

Histopathology of a benign bile duct lesion in the liver: Morphologic mimicker or precursor of intrahepatic cholangiocarcinoma

A bile duct lesion originating from intrahepatic bile ducts is generally regarded as an incidental pathologic finding in liver specimens. However, a recent study on the molecular classification of intrahepatic cholangiocarcinoma has focused on the heterogeneity of this carcinoma and has suggested that the cells of different origins present in the biliary tree may have a major role in the mechanism of oncogenesis. In this review, benign intrahepatic bile duct lesions—regarded in the past as reactive changes or remnant developmental anomalies and now noted to have potential for developing precursor lesions of intrahepatic cholangiocarcinoma—are discussed by focusing on the histopathologic features and its implications in clinical practice.

A histopathological study of liver and biliary remnants in the long-term survivors (>10 years) of cases of biliary atresia.

Context: Biliary atresia (BA) is a destructive process affecting both extraand intra-hepatic bile ducts leading to fibrosis and obliteration of the biliary tree and cirrhosis usually within 2 years. Factors influencing the outcome of portoenterostomy (PE) have not been clearly defined. Aims: Our aim was to identify children with no evidence of liver disease 10 years or more after PE and to compare the pathology of liver and biliary remnants in this group with those associated with poor outcome. Settings and Design: Prospective observational study. Materials and Methods: Wedge biopsies of liver and portal remnants, taken at the time of PE, where available, were reviewed. The parameters studied were - presence of large bile ducts (>150 μ), degree of fibrosis and bile duct proliferation (BDP), presence of ductal plate malformation (DPM) and age at operation. Statistical Analysis Used: Fisher’s exact test with Freeman Halton extension for univariate analysis and Logistic regression analysis as multivariate analysis. Results: Of 68 cases operated between 1995 and 2001, 14 patients survived >10 years and 54 were associated with poor outcome. Large ducts were significantly more in survivors (70% vs. 26%, P = 0.02). DPM was not seen in any of the survivors and was present in 24% of poor outcome group. Fibrosis and BDP were also significantly less among the survivors (P < 0.001, P = 0.03, respectively). The mean ages at operation in the two groups were 66.8 and 89.6 days, respectively. Conclusion: From this study, we feel that lower degree of fibrosis and BDP, absence of DPM, presence of large ducts and younger age at operation were associated with better long-term outcome. Of these, degree of fibrosis was the most significant factor.

Intrahepatic cholangiocarcinoma with predominant ductal plate malformation pattern

No abstract available.

A histopathological study of liver and biliary remnants with clinical outcome in cases of extrahepatic biliary atresia.

Context: The indicators of poor prognosis in cases of extrahepatic biliary atresia (EHBA) continue to remain controversial. Aims: To correlate the histopathological findings of wedge biopsy from liver and tissue obtained from the shaving at the porta hepatis, during hepatic portoenterostomy, with the clinical outcome. Materials and Methods: All cases of EHBA surgically treated in our hospital from 1995 to 2006 have been reviewed. Wedge biopsies of the liver and biopsies from the porta hepatis were analyzed with hemotoxylin-eosin stains and immunohistochemistry. The parameters correlated with clinical outcomes were - presence of large bile ducts ( > 150μm diameter) in the portal tissue plaque, degree of fibrosis (semi-quantitative; graded as mild, moderate and severe), presence of ductal plate malformation (DPM) and age at operation. Results: The proportions of patients with small or large ductal diameter who remained clinically controlled (serum bilirubin < 1.5mg/dl with no evidence of end stage liver failure) were 39% and 66.6% respectively (P=0.44). There was a highly significant correlation between the extent of fibrosis and clinical outcome. Mild, moderate and severe fibrosis resulted in clinical control rates of 78.5%, 34.4% and 24% respectively (P=0.001). Ductal plate malformation was seen in 15% of our cases and was uniformly associated with poor outcome. A non-significant trend towards poorer outcome was seen with increasing age at surgery. Conclusions: Histopathological correl ations with clinical outcome in EHBA have been rarely reported from the Indian subcontinent. A greater degree of fibrosis at the time of hepatic portoenterostomy and presence of ductal plate malformation is associated with a significantly poorer clinical outcome.