Hepatoportal Sclerosis in Childhood: Descriptive Analysis of 12 Patients

Hepatoportal sclerosis (HPS) is defined as sclerosis of portal areas in the absence of cirrhosis. There is little information about HPS in children in the literature. The aim of this study was to describe the clinical presentation, associated disorders, laboratory characteristics and outcome of children who were diagnosed as HPS. This study included 12 children diagnosed as HPS by the Pathology Department between 2005 and 2011. Data were collected from the gastroenterology clinic charts retrospectively, including demographics, presentation characteristics, laboratory data and recent status of patients. Twelve patients were enrolled (6 girls, 6 boys). The median age of patients was 13.5 yr. Median age at the time of biopsy was 11 yr. Four patients had splenomegaly, 3 had esophageal varices, one had hepatopulmonary syndrome and had been transplanted. Smooth muscle antibody was found positive in 4 patients, without autoimmune hepatitis findings in liver biopsy. One patient had celiac disease and another patient had positive celiac disease serology but pathology findings. Another patient had Turner's syndrome. Mean follow-up time was 39 months (3.3 yr) after biopsy. Hepatoportal sclerosis does not necessarily present with portal hypertension in children.

Comparison of interferon monotherapy with interferon-lamivudine combination treatment in children with chronic hepatitis B.

AIM: To compare interferon monotherapy with combination treatment using interferon and lamivudine in children with chronic hepatitis B. METHODS: Data from 65 children who had received either interferon-alpha (5 MU/m2 subcutaneous thrice a week for 6 months; n=35; Group 1) or this dose of interferon-alpha for 6 months with oral lamivudine for one year (4 mg/Kg/day, maximum 100 mg/day; n=30; Group 2) were analyzed retrospectively. Complete response was defined as ALT normalization, HBeAg/anti-HBe seroconversion and HBV DNA clearance. RESULTS: ALT normalization rates were similar in Groups 1 and 2 at the end of interferon treatment (13 [38%] and 16 [52%], respectively), at 12 months (19 [56%] and 18 [58%]) and at 24 months (24 [71%] and 23 [74%]). HBV DNA clearance was more frequently observed at 6 months in Group 2 than in Group 1 (19 [63%] versus 7 [20%]; p=0.01), but not at 12 months (19 [63%] versus 17 [49%]) or at 24 months (20 [67%] versus 21 [60%]). Rate of HBeAg/anti-HBe seroconversion was higher in Group 2 at 12 months (18 [60%] versus 11 [31%]; p< 0.05). Rate of complete response was similar in Groups 1 and 2 at 6 months (5 [14%] and 10 [33%], respectively), 12 months (14 [40%] and 17 [57%]) and 24 months (20 [57%] and 19 [63%]). CONCLUSION: Although lamivudine and interferon combination achieved higher initial rates of HBV DNA loss and HBeAg/anti-HBe seroconversion than interferon alone, the final response rates were similar with the two treatments. The combination treatment is therefore not indicated for chronic hepatitis B in children.