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Resting energy expenditure is increased in infants and children with extrahepatic biliary atresia
A Pierro1, B Koletzko, V Carnielli
1Department of Surgery, University of Toronto, Ontario, Canada.
Insights
Children with extrahepatic biliary atresia (EHBA) experience significantly higher energy expenditure and impaired fat metabolism, leading to malnutrition. Liver dysfunction in these children profoundly impacts energy and macronutrient balance.
Area of Science:
- Pediatric Gastroenterology
- Metabolic Disorders
- Hepatology
Background:
- Liver dysfunction in children can disrupt normal metabolic processes.
- Extrahepatic biliary atresia (EHBA) is a severe condition affecting infants and children.
- Understanding metabolic derangements is crucial for managing pediatric liver disease.
Purpose of the Study:
- To investigate the effects of liver dysfunction in children with EHBA on energy and macronutrient homeostasis.
- To characterize the metabolic alterations contributing to malnutrition in pediatric liver disease.
Main Methods:
- Performed 13 metabolic studies in 11 pediatric patients with EHBA.
- Utilized nutritional balance, indirect calorimetry, anthropometry, and liver function tests.
- Analyzed energy expenditure, substrate oxidation, and nutrient balance.
Main Results:
- Patients exhibited 29% higher energy expenditure (68 kcal/kg/d) and significant energy loss via stool fat (64%).
- Protein oxidation was increased (2g/kg/d), leading to near-zero nitrogen balance, with some patients oxidizing endogenous protein.
- Diminished net lipid oxidation and altered carbohydrate metabolism were observed, with higher serum aspartate aminotransferase correlating with lower fat oxidation.
Conclusions:
- Markedly increased energy expenditure contributes significantly to the malnutrition observed in EHBA patients.
- Severe liver disease in children profoundly affects carbohydrate, fat, and protein metabolism, inducing protein-energy malnutrition.
- This study provides the first characterization of these metabolic disturbances in pediatric liver disease.
Abstract:
To determine if liver dysfunction in children affects energy and macronutrient homeostasis, we performed 13 metabolic studies in 11 patients (age, 17.8 +/- 5.9 months [mean +/- SEM]) with extrahepatic biliary atresia (EHBA). Nutritional balance, indirect calorimetry, anthropometry, and biochemical liver function tests were utilised. Sixty-four percent of the energy losses were in the form of stool fat. Energy expenditure (68 kcal/kg/d) was 29% higher than normal (P less than 0.0025). Only one third of the metabolisable energy intake (37 kcal/kg/d) was stored in the body for new tissue synthesis. In spite of the bountiful protein intake for age, the increased protein oxidation (2g/kg/d) resulted in a virtually zero mean nitrogen balance. In addition, four patients oxidised endogenous protein as well. The respiratory quotient was 0.96, and did not change significantly between pre- and post-meal measurements, suggesting a predominant utilisation of carbohydrate for energy metabolism. Net lipid oxidation was severely diminished. We found that the higher the serum aspartate aminotransferase level (previously named SGOT), the lower the net fat oxidation, and the higher the conversion of glucose to fat. These data suggest that markedly increased energy expenditure contributes to the malnutrition of patients with EHBA. We characterised for the first time how severe liver disease in infants and children affects carbohydrate, fat, and protein metabolism, thus inducing protein-energy malnutrition.