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Background: Neonatal cholestasis syndrome is considered as a major challenge in pediatric practice. This study was undertaken to investigate the value of morphometric assessment of hepatic fibrosis in early diagnosis of biliary atresia., Methods: We studied liver biopsy specimens from 53 patients with neonatal cholestasis. The patients were assigned to two groups: group 1 (25 patients with biliary atresia) and group 2 (28 patients with non-obstructive cholestasis). Morphometric assessment of fibrosis was performed for all biopsies; in addition, another twelve histological parameters were estimated and scored on a scale of 0 to 4. Biopsies of infants aged 60 days or younger were characterized and analyzed separately., Results: Morphometric value of fibrosis was significantly higher in group 1 than in group 2 (16.8 ± 8.4% vs. 5.9 ± 2.3%, respectively; P<0.001). By multiple regression analysis, bile ductular plugs, morphometric assessment of fibrosis, rosetting, portal tract inflammation and pattern of cholestasis were found to be significant in discriminating the two groups. In infants aged 60 days or younger, a cutoff value for morphometric assessment of fibrosis of 7.5% was the discriminating point between the two groups with a sensitivity of 80% and a specificity of 84%., Conclusion: Morphometric assessment of hepatic fibrosis could enhance the value of liver biopsy in early diagnosis of biliary atresia.

Background: Plasma endothelin-1 (ET-1) is a potent vasoconstrictor peptide involved in the pathogenesis of several disorders. Endothelin-1 concentrations are increased in adult patients with cirrhosis. However, little is known about ET-1 concentrations in children with cirrhosis., Methods: Radioimmune assay was used to measure plasma ET-1 concentrations in 19 children with cirrhosis (8 patients with ascites, and 11 without ascites), and 11 age- and sex-matched healthy children. The plasma ET-1 concentrations were correlated with the mean blood pressure, creatinine clearance, and severity of portal hypertension, as measured by portal flow volume and portal flow velocity., Results: Patients with cirrhosis and ascites had increased plasma ET-1 concentrations compared with patients who did not have ascites (6.8 pg/mL +/- 0.62 pg/mL vs. 4.6 pg/mL +/- 0.35 pg/mL; mean +/- SEM; < 0.01) and controls (3.6 pg/mL +/- 0.27 pg/mL; mean +/- SEM; < 0.0005). Plasma ET-1 concentrations were higher in patients with cirrhosis who did not have ascites compared with controls ( < 0.005). No significant differences were observed between concentrations of the patients with cholestasis and those without cholestasis (5.4 pg/mL +/- 0.52 pg/mL vs. 5.2 +/- 0.32 pg/mL; mean +/- SEM; = 0.1). Plasma ET-1 concentrations correlated positively with the mean blood pressure ( = 0.58; < 0.05) and negatively with renal function, as measured by creatinine clearance ( = -0.7; <0.005). However, no correlation was detected between ET-1 concentrations and portal flow volume ( = -0.02; = 0.4) or portal flow velocity ( = -0.16; = 0.4)., Conclusions: Plasma ET-1 concentrations are increased in children with cirrhosis, with or without ascites, compared with controls. Patients with cirrhosis and ascites have increased ET-1 concentrations compared with those without ascites. The degree of increase does not relate to the severity of portal hypertension. This increase tends to maintain systemic blood pressure but is associated with a decrease in renal function.

Intercellular adhesion molecule-I (ICAM-I) is a member of the immunoglobulin supergene family. It is expressed on the surface membrane of cells of multiple lineages at sites of inflammation. A soluble form of ICAM (sICAM-I) comprising the five extracellular Ig-like domains of ICAM-I has been detected in human serum and has been found to be increased in a variety of acute and chronic liver disorders. However, little is known about sICAM-I levels in children with chronic liver disease. Therefore, we measured sICAM-I in 23 children with chronic hepatitis, 14 children with cirrhosis, and 10 age- and sex-matched normal children by commercially available ELISA. We also correlated the sICAM-I levels with the histological activity index (HAI) score as determined from liver biopsies. Patients with chronic hepatitis had higher sICAM-I levels compared to controls (723 +/- 272 ng/ml vs 282 +/- 43 ng/ml, mean +/- SD; P < 0.05). sICAM-I levels were also higher in patients with cirrhosis compared to controls (630 +/- 218 ng, mean +/- SD; P < 0.05). However, there was no significant difference between sICAM levels in patients with chronic hepatitis and cirrhosis. A significant correlation was found between the ICAM-I level and the histological activity index score in patients with chronic hepatitis (r = 0.58; P < 0.001) and in patients with cirrhosis (r = 0.7; P < 0.001). We also found that by using the cutoff level of 346 ng/ml, sICAM-I can be used as a screening test with high specificity (100%) and sensitivity (94%) to differentiate children with chronic liver disease from normal children. We conclude that sICAM is increased in children with chronic hepatitis and cirrhosis compared to controls. The degree of elevation correlates with the HAI score. sICAM may be used as a marker of the disease activity and may provide diagnostic and prognostic information in children with chronic liver disease. However, this needs to be further studied.