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Updated: Aug 12, 2026

Invasive Hemodynamic Characterization of the Portal-hypertensive Syndrome in Cirrhotic Rats
Published on: August 1, 2018
[Carbohydrate metabolism enzymes in children with extrahepatic portal hypertension]
Insights
Extrahepatic portal hypertension in children significantly reduces liver glycogen and glucose-6-phosphatase activity, impacting blood glucose homeostasis. These biochemical changes impair the body's adaptive capacity, crucial for therapeutic planning.
Area of Science:
- Biochemistry
- Pediatric Gastroenterology
- Hepatology
Context:
- Extrahepatic portal hypertension (EHPHT) is a condition affecting children, leading to impaired liver function.
- Understanding the biochemical alterations in the liver is crucial for managing EHPHT.
- Previous research has not fully elucidated the specific enzymatic changes in pediatric EHPHT.
Purpose:
- To investigate glycogen and protein concentrations and the activities of key liver glycogen metabolic enzymes in children with EHPHT.
- To identify characteristic biochemical markers associated with EHPHT in pediatric patients.
- To explore correlations between enzyme activities, glycogen levels, and clinical outcomes.
Summary:
- This study analyzed liver glycogen metabolic enzymes in 22 children (4-15 years) with EHPHT.
- Key findings include reduced phosphorylase alpha activity, decreased glycogen concentration, and diminished glucose-6-phosphatase activity.
- Strong correlations were observed between these enzymatic changes and EHPHT, particularly involving phosphorylase alpha and glucose-6-phosphatase.
Impact:
- Impaired liver blood flow in EHPHT injures glucose homeostasis maintenance, a primary liver function.
- Reduced blood glucose regulation diminishes the body's adaptive potential in affected children.
- These findings highlight the importance of considering these biochemical deficits in therapeutic strategies for pediatric EHPHT.
Abstract:
Glycogen and protein concentrations and the activities of liver glycogen metabolic enzymes were measured in 22 children aged 4 to 15, suffering from extrahepatic portal hypertension. Glucose-6-phosphatase, amylo-1,6-glucosidase, fructose-1,6-diphosphatase, phosphorylases alpha and beta, phosphoglucomutase, and phosphohexose isomerase levels were analyzed. Liver biopsy specimens obtained by surgical marginal biopsy were used in the study. No or drastic reduction of phosphorylase alpha activity and reduction of glycogen concentration and glucose-phosphatase activity were found characteristic of extrahepatic hypertension. Analysis of correlations of the findings has demonstrated a medium correlation in 4 cases and a strong correlation between the findings in 1 case, the possibility being estimated as 0.95-0.99. The highest number of correlations was observed with phosphorylase alpha and glucose-6-phosphatase (3 correlations). Liver blood stream impairments result in injury to one of its main biochemical functions, i.e., the maintenance of blood glucose homeostasis, this leading to reduction of the adaptation potential of the body; this should be borne in mind when planning therapeutic measures for patients with extrahepatic hypertension.
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