10 Cholestasis and end-stage liver disease

https://doi.org/10.1016/S0950-3528(98)90010-0Get rights and content

Abstract

Protein-energy malnutrition is an inevitable consequence of chronic liver disease, particularly in the developing infant. Severe malnutrition with loss of fat stores and muscle wasting affects between 60% and 80% of infants with liver disease (Beath, 1993a; Holt et al, 1997). Reduced energy intake secondary to anorexia, vomiting and fat malabsorption, in association with a disordered metabolism of carbohydrate and protein, increased energy requirements and vitamin and mineral deficiencies, contributes towards growth failure. Reversal of malnutrition is one of the key aims of liver transplantation and is achieved in the majority of long-term survivors. The aetiology of persistent growth failure posttransplantation is multifactorial and is related to pre-operative malnutrition, glucocorticoid administration, feeding problems and post-operative complications. Strategies to prevent pre- and post-transplant growth failure include early referral for liver transplantation and a multidisciplinary approach to nutritional support, which may increase survival and improve the quality of life and outcome of liver transplantation.

References (58)

  • M Makrides et al.

    Are long-chain polyunsaturated fatty acids essential nutrients in infancy?

    Lancet

    (1995)
  • A Pierro et al.

    Resting energy expenditure is increased in infants with extra hepatic biliary atresia and cirrhosis

    Journal of Pediatric Surgery

    (1989)
  • SM Protheroe et al.

    Can measurement of dietary-induced thermogenesis (DIT) predict response to nutritional intervention in infants with liver disease?

    Clinical Nutrition

    (1996)
  • S Sarna et al.

    Mechanisms and treatment of growth retardation in children with liver transplants

  • WS Andrews et al.

    Steroid withdrawal after paediatric liver transplantation

  • S Beath et al.

    Long chain triacyglycerol malabsorption and pancreatic function in children with protein energy malnutrition complicating severe liver disease

  • SV Beath et al.

    Successful liver transplantation in babies under one year

    British Medical Journal

    (1993)
  • SV Beath et al.

    Nutritional support in liver disease

    Archives of Disease in Childhood

    (1993)
  • S Beath et al.

    Improving outcome of liver transplantation in babies less than I year

  • JC Bucuvalas et al.

    Growth hormone insensitivity in children with biliary atresia

    Journal of Paediatric Gastroenterology and Nutrition

    (1996)
  • V Burke et al.

    Medium chain triglyceride diet: its use in treatment of liver disease

    British Medical Journal

    (1966)
  • J Cardona et al.

    Liver transplantation in children with Alagille syndrome—a study of twelve cases

    Transplantation

    (1995)
  • SE Carlson et al.

    First year growth of preterm infants fed standard compared to marine oil n-3 supplemented formula

    Lipids

    (1992)
  • VP Carnielli et al.

    Structural position and amount of palmitic acid in infant formulas: effects on fat, fatty acid and mineral balance

    Journal of Paediatric Gastroenterology and Nutrition

    (1996)
  • CPJ Charlton et al.

    The use of enteral feeding in the dietary management of children with chronic liver disease

    Archives of Disease in Childhood

    (1992)
  • SE Chin et al.

    Survival, growth and quality of life in children after orthotopic liver transplantation: a 5 year experience

    Journal of Pediatrics and Child Health

    (1991)
  • SKF Chong et al.

    Exocrine pancreatic insufficiency in syndromic paucity of interlobular bile ducts

    Journal of Pediatric Gastroenterology and Nutrition

    (1989)
  • S Dunn et al.

    Monotherapy with cyclosporine for chronic immunosuppression in paediatric liver transplant recipients

    Transplantation

    (1994)
  • PJ Garlick et al.

    Amino acid infusion increases the sensitivity of muscle protein synthesis in vivo to insulin

    Biochemical Journal

    (1988)
  • Cited by (20)

    • Liver Transplantation in Children

      2020, Pediatric Gastrointestinal and Liver Disease, Sixth Edition
    • Expression of fibroblast growth factor 21 in patients with biliary atresia

      2016, Cytokine
      Citation Excerpt :

      BA (biliary atresia) is the most common cause of cholestatic liver injury, which could progress to fibrosis, cirrhosis and end-stage liver disease (ESLD) [1,2]. ESLD is the consequence of cholestatic liver injury leading to irreversible liver dysfunction [3,4]. The presenting symptoms of ESLD include impaired synthesis of serum proteins and coagulation factors (quantified by INR), imbalance of glucose, lipid and protein metabolism, and impaired bile excretion [5].

    • Liver Transplantation in Children

      2011, Pediatric Gastrointestinal and Liver Disease
    • Liver Transplantation in Children

      2010, Pediatric Gastrointestinal and Liver Disease
    • Liver Failure and Transplantation

      2006, Pediatric Gastrointestinal and Liver Disease: Pathophysiology, Diagnosis, Management
    • Liver Failure and Transplantation

      2006, Pediatric Gastrointestinal and Liver Disease
    View all citing articles on Scopus
    View full text