Resting energy expenditure is increased in infants and children with extrahepatic biliary atresia

Agostino Pierro, Bert Koletzko, Virgilio Carnielli, Riccardo A. Superina, Eva A. Roberts, Robert M. Filler, John Smith, Tibor Heim*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

87 Scopus citations


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. Sixtyfour percent of the energy losses were in the form of stool fat. Energy expenditure (68 kcal/kd/d) was 29% higher than normal (P<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.

Original languageEnglish (US)
Pages (from-to)534-538
Number of pages5
JournalJournal of pediatric surgery
Issue number6
StatePublished - Jun 1989


  • Extrahepatic biliary atresia
  • energy metabolism
  • substrate utilisation

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health


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