Nutritional support in children with end-stage liver disease: a randomized crossover trial of a branched-chain amino acid supplement

Abstract

Malnutrition is common in children with end-stage liver disease (ESLD) awaiting orthotopic liver transplantation (OLT), and nutritional support is assuming an important role in preoperative management. To evaluate preoperative nutritional therapy, 19 children (median age 1.25 y) with ESLD awaiting OLT were prospectively studied. Two high-energy, isoenergetic and isonitrogenous nutritional formulations delivered nasogastrically were compared: a branched-chain amino acid (BCAA)-enriched semielemental formulation and a matched standard semielemental formation. Twelve of 19 patients completed a randomized controlled study before OLT and 10 of 19 completed a full crossover study. Improvements in weight and height occurred during the BCAA supplements, with no statistical change on the standard formulation. Significant increases in total body potassium, midupper arm circumference, and subscapular skinfold thickness occurred during the BCAA supplements, whereas no significant changes occurred during the standard formulation period. Significantly fewer albumin infusions were required during the BCAA supplement. These findings suggest that BCAA-enriched formulas have advantages over standard semielemental formulas in improving nutritional status in children with ESLD, and are deserving of wider application and study.

Identification of Urinary Biomarkers Useful for Distinguishing a Difference in Mechanism of Toxicity in Rat Model of Cholestasis

“…Children with mild-moderate chronic cholestatic liver disease had decreased plasma concentrations of branched-chain amino acids in the presence of increased requirements for total branched-chain amino acids [46,47], suggesting that decreases in plasma branched-chain amino acids levels may be reflective of increases in branched-chain amino acids requirements. Correspondingly, urinary branched-chain amino acids levels are considered to be decreased.…”

Nutrición parenteral en situaciones clínicas especiales

“…En niños la administración de suplementos orales no ha mostrado beneficios claros (55). En adultos, una reciente revisión Cochrane, que incluye siete ensayos clínicos que evalúan la administración intravenosa de BCAA en pacientes con encefalopatía hepática, muestra beneficios sobre la evolución clínica de la encefalopatía, aunque no encuentra diferencias en la mortalidad, calidad de vida o parámetros nutricionales (56).…”

Nutritional Status and Liver Transplantation

“…Chin et al compared two pre-operative nutritional supplementations (a BCAA-enriched versus a matched standard semi-elemental formulation) in 19 children with end-stage liver disease waiting for liver transplant. 59 Twelve patients completed the study before being transplanted and 10 completed a full crossover study. Both regimens improved children's weight and height, and BCAA supplements caused a significant increase of mid-arm circumference and subscapular skinfold thickness.…”