Hepatoportal sclerosis in childhood: descriptive analysis of 12 patients
Mustafa Serdar Cantez1, Nelgin Gerenli, Vildan Ertekin
1Istanbul University, Istanbul Faculty of Medicine, Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology and Nutrition, Istanbul, Turkey.
Insights
Hepatoportal sclerosis (HPS) in children is rare, often lacking typical portal hypertension signs. This study details clinical features and outcomes in pediatric HPS cases.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Pathology
Background:
- Hepatoportal sclerosis (HPS) is characterized by portal area sclerosis without cirrhosis.
- Limited data exists on HPS in pediatric populations.
- Understanding HPS in children is crucial for diagnosis and management.
Purpose of the Study:
- To elucidate the clinical presentation of pediatric HPS.
- To identify associated disorders and laboratory findings in children with HPS.
- To describe the outcomes of children diagnosed with HPS.
Main Methods:
- Retrospective chart review of 12 children diagnosed with HPS between 2005 and 2011.
- Data collection included demographics, clinical presentation, laboratory results, and patient outcomes.
- Histopathological diagnosis of HPS was confirmed by the Pathology Department.
Main Results:
- Twelve pediatric patients (6 boys, 6 girls) were included, with a median age of 13.5 years at enrollment.
- Associated conditions included splenomegaly (4 patients), esophageal varices (3 patients), and hepatopulmonary syndrome (1 patient requiring transplant).
- Positive smooth muscle antibodies were noted in 4 patients without concurrent autoimmune hepatitis; celiac disease and Turner's syndrome were also observed.
Conclusions:
- Hepatoportal sclerosis in children may not always manifest with overt signs of portal hypertension.
- Pediatric HPS can be associated with autoimmune markers and other specific syndromes.
- Further research is needed to understand the long-term implications and management strategies for HPS in children.
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.
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