title: Hepatoportal sclerosis in childhood: descriptive analysis of 12 patients.
abstract: 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.
authors.affiliation: Istanbul University, Istanbul Faculty of Medicine, Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology and Nutrition, Istanbul, Turkey.
authors.forename: Mustafa Serdar
authors.initials: MS
authors.lastname: Cantez
authors.forename: Nelgin
authors.initials: N
authors.lastname: Gerenli
authors.forename: Vildan
authors.initials: V
authors.lastname: Ertekin
authors.forename: Mine
authors.initials: M
authors.lastname: Güllüoğlu
authors.forename: Özlem
authors.initials: Ö
authors.lastname: Durmaz
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D005762:Gastroenterology; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D008099:Liver / Q000473:pathology; D008107:Liver Diseases / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D008297:Male; D011169:Portal Vein / Q000473:pathology; D012189:Retrospective Studies; D012598:Sclerosis / Q000175:diagnosis* / Q000473:pathology*
keywords: Child; Hepatoportal Sclerosis; Idiopathic Noncirrhotic Portal Hypertension